Xanthogranulomatous cholecystitis (XGC) is a rare type of chronic cholecystitis characterized by severe proliferative fibrosis with infiltration of macrophages and foamy cells in the gallbladder wall. Since XGC and gallbladder carcinoma have similar clinical manifestations and radiological features, XGC is often misdiagnosed as gallbladder carcinoma in clinical practice, which leads to unnecessary extensive surgical resection and has an adverse effect on patients. At present, the preoperative diagnosis of XGC is still based on imaging results (ultrasound, computed tomography, and magnetic resonance imaging), and a definite diagnosis of this disease relies on intraoperative frozen biopsy or postoperative pathological examination. Meanwhile, XGC should be differentiated from gallbladder adenomyomatosis, gallbladder carcinoma, and gallbladder actinomycosis. Laparotomy or laparoscopic cholecystectomy is the major method for the treatment of XGC, but laparoscopic cholecystectomy is associated with a longer time of operation, more complications, and a higher rate of conversion to laparotomy. Therefore, surgeons are facing difficulties in preoperative diagnosis and intraoperative decision-making process of XGC.