Background and Aims Pancreatic hepatoid adenocarcinoma (PHC) is a rare and aggressive adenocarcinoma with hepatocellular carcinoma (HCC)-like characteristics, primarily found in the tail of the pancreas. It has a low incidence rate, high malignancy, strong invasiveness, early propensity for lymphatic and distant metastasis, rapid disease progression, and poor prognosis. Clinical and radiological features of PHC lack specificity, making preoperative diagnosis challenging. However, most patients exhibit elevated alpha-fetoprotein (AFP) levels, which serves as a diagnostic clue. Nevertheless, the definitive diagnosis of the disease requires pathological examination. This article retrospectively analyzes and summarizes the clinical characteristics and treatment process of one patient with PHC and multiple intra-abdominal organ metastases, as well as reviews relevant literature from both domestic and international sources to enhance clinicians' understanding of PHC and continually improve treatment strategies for the disease.Methods The clinical data and treatment process of one patient with PHC and multiple intra-abdominal organ metastases treated in the Department of Hepatobiliary and Pancreatic Surgery at the Second Affiliated Hospital of Kunming Medical University were retrospectively analyzed. The pathogenesis, clinical characteristics, diagnostic methods, and disease treatment options were analyzed and summarized in combination with a review of relevant domestic and international literature.Results The patient was a 63-year-old male admitted to the hospital due to abdominal distension, abdominal pain, fatigue, and poor appetite for over three months. Examination revealed space-occupying lesions in multiple organs, including the liver, pancreas, spleen, and stomach, with the nature of the lesions yet to be determined. The patient underwent palliative tumor resection surgery. Postoperative pathological results supported the diagnosis of PHC, with a solid type and a histological grade of 3. Three months after surgery, intrahepatic metastases were detected, and the patient underwent hepatic arterial infusion chemotherapy with oxaliplatin (130 mg), fluorouracil (200 mg), and embolization therapy. Subsequently, the lesions metastasized to the lungs, and the patient died 10 months after surgery due to multiple organ failure.Conclusion PHC is a rare, poorly understood adenocarcinoma with HCC-like differentiation, lacking typical clinical and radiological features. It exhibits high malignancy early lymphatic and distant metastasis and is often diagnosed in advanced stages, and the opportunity for radical surgical resection has already been lost. The pathogenesis of PHC remains unclear, but it presents with characteristic HCC-like differentiation and often shows high levels of AFP and HCC immunohistochemical markers, making pathological examination the gold standard for diagnosis. Currently, there is no consensus on diagnosing and treating PHC at home and abroad. Aggressive surgical treatment is recommended for patients eligible for surgical resection to improve prognosis. However, adjuvant therapy should be considered for those who cannot undergo radical resection. Radiotherapy is generally considered ineffective, and chemotherapy is an independent prognostic factor, though the optimal chemotherapy regimen remains debated. Therefore, if conditions permit, an appropriate chemotherapy regimen can be selected after biopsy diagnosis to improve patients' objective remission rate. For those with an unclear diagnosis, treatment can follow protocols used for gastrointestinal system diseases.