Background and Aims Hepatic angiomyolipoma (HAML) is a rare benign mesenchymal tumor of the liver, with no specific imaging features and low preoperative diagnosis rate. It is frequently misdiagnosed as hepatocellular carcinoma (HCC) or other hepatic benign tumors. This article reports the diagnosis and treatment process of 19 patients with HAML, so as to provide a reference and guidance for clinical practice.Methods The clinical data of 19 cases with HAML treated from January 2011 to December 2019 were retrospectively analyzed. Of the patients, 12 cases were females and 7 cases were males, with the age ranging from 28 to 61 years; lesion was found by health maintenance examination in 14 cases without clinical symptoms, 3 cases had abdominal pain, one case had intermittent abdominal distention, and one case had abdominal pain and diarrhea; one case was complicated with chronic hepatitis B and pulmonary abscess, one case was complicated with liver cirrhosis in compensatory period. All the 19 patients did not have angiomyolipoma of other organs such as kidney and lung, and none of them had tuberous sclerosis. The diameter of the tumors ranged from 1.3 to 12 cm, with an average diameter of (4.6±2.2) cm. The levels of AFP, CA19-9 and CEA were all within the normal range. Seven patients (36.8%) were diagnosed as HCC before operation, and 12 patients (63.2%) were considered as hepatic benign tumors that included 7 cases were diagnosed as hepatic adenoma, 2 cases were diagnosed as inflammatory pseudotumor, one case was diagnosed as hepatic cavernous hemangioma, one case was diagnosed as hepatic focal nodular hyperplasia, and one case was identified as HAML by preoperative ultrasound guided liver biopsy pathology.Results All patients underwent surgical treatment, including hepatic resection in 18 patients and ultrasound guided liver tumor biopsy and percutaneous puncture radiofrequency ablation of the liver tumor in one patient. The average operative time was (172.7±80.4) min, intraoperative blood loss was (456.6±528.1) mL, and length of hospital stay was (9.8±2.7) d. HAML was confirmed in all the 19 patients by pathological examination, in which seven cases were epithelioid angiomyolipoma. HMB-45 and SMA were positive in immunohistochemical staining. All patients recovered well after operation, and no complications such as liver failure, abdominal bleeding and bile leakage as well as death occurred. All patients were followed up, and no tumor recurrence and metastasis were found. All patients had good quality of life.Conclusions HAML belongs to benign tumor, and is difficult to diagnose before operation. Some patients were easily misdiagnosed as HCC. The final diagnosis depends on pathological and immunohistochemical analysis. Surgical resection is a safe and effective treatment option with good prognosis.