Background and Aims Hepatic angiomyolipoma (HAML) is a rare benign liver tumor that can be difficult to distinguish from other benign and malignant liver tumors. The clinical diagnosis is challenging, and the treatment strategies and prognosis remain unclear. This study was performed to explore the clinical, imaging, and pathological characteristics, as well as the treatment strategies and prognosis of HAML, to provide a reference for its clinical diagnosis and treatment.Methods The clinical, pathological, imaging, and follow-up data of 46 cases of HAML treated in the Department of Hepatobiliary and Pancreatic Surgery of the Second Xiangya Hospital of Central South University from January 2012 to December 2023 were retrospectively analyzed.Results Among the 46 HAML patients, 12 were male (26.1%) and 34 were female (73.9%), with an age range of 20 to 70 years (median age of 46 years). Twenty-nine cases were asymptomatic at onset, 17 presented with abdominal discomfort, 6 had concurrent hepatitis B, and 7 had concurrent renal angiomyolipoma (RAML). No cases were associated with tuberous sclerosis. Tumor markers (AFP, CEA, CA19-9, and abnormal prothrombin Ⅱ) were not significantly abnormal. Based on clinical data from 4 HAML patients, the average growth rate of HAML was 0.46 cm/year, with a median growth rate of 0.39 cm/year. Imaging characteristics were diverse, with the accuracy rates of preoperative imaging diagnosis of HAML being 3.8% for ultrasound, 34.1% for CT, and 40.9% for MRI. Among the 46 samples, 26 cases (56.5%) had left liver lesions, 18 cases (39.1%) had right liver lesions, and 2 cases (4.3%) had caudate lobe lesions. One case had multiple lesions confined to the left liver, and the remaining 45 cases had single lesions. Tumor diameters ranged from 1 to 15 cm, with a median diameter of 4.3 cm. Pathological results indicated that 41 cases were benign, while 5 cases were malignant; 10 cases were epithelioid HAML. Immunohistochemistry showed 100% positivity for HMB-45 and Melan-A, 95.1% for SMA, 89.7% for CD34, and 54.5% for S-100. Follow-up ranged from 0.25 to 12 years, with 16 cases lost to follow-up. One case recurred 19 months after the operation and 2 years later died of lung adenocarcinoma (confirmed by postoperative pathology at our center), and another case died of lung cancer 5 years after the operation (confirmed by pathology at an external hospital). Both deaths were unrelated to HAML. The remaining 28 patients were healthy with no recurrence or metastasis.Conclusion HAML predominantly occurs in middle-aged women, often without apparent symptoms, and grows slowly, making it prone to misdiagnosis through imaging. Pathological diagnosis is the gold standard for HAML, with HMB-45 and Melan-A positivity being specific indicators. The majority of HAML cases are benign, with a minority being malignant. Overall prognosis is good, but epithelioid and malignant HAML have a risk of recurrence, warranting active follow-up. Surgical resection is an effective treatment for HAML.