Purpose Our primary objective was to investigate the clinical features, diagnosis, treatment and prognosis of hepatic perivascular epithelioid cell tumours (PEComa).Patients and Methods Thirty-five cases of pathologically proven hepatic PEComa that were treated in the Department of Hepatobiliary Center of the First Affiliated Hospital of Nanjing Medical University from January 2008 to February 2019 were retrospectively analysed, and the literature was reviewed.Results Twenty-nine females and 6 males were included in this study. The mean age of these patients was 48.0 years (range, 21-75 years). Thirteen patients complained of upper abdominal pain and discomfort, but the tumours in the other patients were identified by performing an imaging examination. Hepatic PEComas tended to occur in the right lobe of the liver (20 cases in the right lobe, 13 in the left lobe and 2 in the caudate lobe). Two cases were characterized by multiple tumours, and the remaining cases were single lesions (range, 1.2-12 cm). Only 8 cases were correctly diagnosed by the preoperative imaging examination, and the correct diagnosis rate was only 22.9%. The postoperative immunohistochemistry analysis showed that hepatic PEComas are positive for human melanoma black 45 (HMB-45), Melan-A and smooth muscle actin (SMA), with the exception of 1 case that was negative for Melan-A. All patients undergoing an operation accepted regular follow-up, and the average time was 66.5 months (range, 3-132 months). Two patients who experienced tumour recurrence and 1 patient who died due to cardiovascular disease, but the remaining patients showed no evidence of tumour recurrence or metastasis during the follow-up period.Conclusions Hepatic PEComa is a rare type of tumour that mainly occurs in young and middle-aged women. The lack of clinical manifestations and imaging findings increases the difficulty of determining a preoperative diagnosis, which mainly depends on the pathological examinations. Surgery is currently the only effective treatment, and long-term clinical follow-up is necessary due to the aggressive behaviour and relapse of hepatic PEComa in some cases.