ObjectiveTo analyze the clinical features and diagnosis of hepatic angiomyolipoma patients, and to investigate its treatment strategy. MethodsThe clinical data of 79 patients with pathologically-confirmed hepatic angiomyolipoma, who were treated in the Eastern Hepatobiliary Surgery Hospital during 1992-2006, were retrospectively reviewed. ResultsThe patients included 58 women and 21 men with a median age of (43±8.14) years old (ranging 17 to 69 years). None of the patients was found complicated with renal angiomyolipoma or tuberous sclerosis complex. Twenty-five patients were symptomatic on presentation. All the patients had a single hepatic lesion, with the tumor size ranging 1.0-25 cm and a median of (6.1±4.08) cm. Fifty-three tumors were located in the right lobe, 22 in the left lobe, and 4 in the caudate lobe. Preoperative diagnoses were correctly made in 41 (52%) patients. Spontaneous rupture of tumor occurred in 1 patient. All the patients underwent surgical treatment without operative mortality and serious morbidity. The patients were regularly followed-up for 3-13 years. Tumor recurrence occurred in one patient 6 years after the surgery, and the patient died 1 year later. ConclusionHepatic angiomyolipoma has no specific clinical signs and symptoms. Comprehensive imaging studies can help to make correct diagnosis, but the differential diagnosis is difficult, especially for small tumors with diameter less than 5 cm. The final diagnosis depends on pathologic examination and HMB-45 special staining. Surgical resection is safe and effective for the disease. Attention should be paid to the potential of malignant transformation and risk of rupture as a life-threatening complication. Early operation is needed once a diagnosis is made, and the patients should be followed up after operation.