Objective: To evaluate the efficacy and safety of laparoscopic surgery for perivascular epithelioid cell tumor (PEComa). Methods: The clinical data of 42 patients with hepatic PEComa diagnosed by pathology in Hunan Provincial People's Hospital from September 2012 to September 2021 were retrospectively analyzed. The patients were divided into the endoscopic group and the open group according to surgical methods. Statistical software was used to compare the differences in operation time, intraoperative blood loss, postoperative hospital stay, postoperative pathological data and incidence of complications between the two groups. Results: There were 27 cases in the endoscopic group and 15 cases in the open group. In the endoscopic group, there were 5 males and 22 females, aged (40.0±10.4) years. In the open group, there were 5 males and 10 females, aged (44.5±12.6) years. The operative time of the endoscopic group and the open group was (239±156.2) min and (348±103.0) min, and the postoperative hospital stay was (8.2±2.4) d and (13.7±4.9) d, respectively, the endoscopic group was significantly better than the open group, and the difference was statistically significant (P<0.05). There was no significant difference in intraoperative blood loss, tumor benign and malignant, tumor site, tumor diameter, Ki67 index, postoperative complications such as biliary leakage, incision infection and pleural effusion (P>0.05). During the follow-up period of 2-103 months, one case was lost to follow-up, two cases died in the endoscopic group,one case died in the open group. The 5-year overall survival rate (OS) and disease-free survival rate (DFS) were 80.8% and 83.0%, respectively. Meanwhile,. The 5-year OS and DFS were both 92.3%, the difference was not statistically significant (P>0.05). Conclusions: Laparoscopic treatment of hepatic PEComa has the advantages of short operation time and short postoperative hospital stay, and the incidence of complications, 5-year OS and DFS are not significantly different from that of the open group.