Background: Xanthogranulomatous cholecystitis (XGC) is a rare benign chronic inflammatory disease of the gallbladder that often presents as cholecystitis and most of the times requires surgical management. In addition, distinguishing XGC from gallbladder cancer preoperatively is still a challenge. The aim of the present systematic review was to outline the clinical presentation and surgical approach of XGC. Data sources: The present systematic review was designed using the PRISMA and AMSTAR guidelines. We searched MEDLINE, Scopus, Clinicaltrials.gov, EMBASE, Cochrane Central Register of Controlled Trials (CENTRAL) and Google Scholar databases from inception until June 2020. Results: The laparoscopic cholecystectomy rate (34％) was almost equal to the open cholecystectomy rate (47％) for XGC. An important conversion rate (35％) was observed as well. The XGC cases treated by surgery were associated with low mortality (0.3％), limited intraoperative blood loss (58-270 mL), low complication rates (2％–6％), along with extended operative time (82.6–120 minutes for laparoscopic and 59.6–240 minutes for open cholecystectomy) and hospital stay (3–9 days after laparoscopic and 8.3–18 days after open cholecystectomy). Intraoperative findings during cholecystectomies for XGC included empyema or Mirizzi syndrome. In addition, complex surgical procedures, like wedge hepatic resections and bile duct excision were required during operations for XGC. Conclusions: XGC seemed to be a rare, benign inflammatory disease that presents similar features as gallbladder cancer. The mortality and complication rates of XGC were low, despite the complex surgical procedures that might be required in some cases.