Xanthogranulomatous cholecystitis (XGC) is a rare form of cholecystitis, characterized by the presence of xanthogranuloma, prominent yellow structures within the gallbladder wall that is very often lithiasic. When XGC presents in its pseudo-tumoral form with occasional adjacent organ involvement, it can mimic gallbladder carcinoma (GBC). The etiopathogenesis of XGC is inflammatory destruction of Rokitansky-Aschoff sinuses containing biliary and cholesterol pigments within the gallbladder wall; this leads to a florid granulomatous histiocytic inflammatory reaction. The prevalence ranges from 1.3% to 8.8% of all cholecystectomies and varies from country to country; XGC occurs predominantly in patients over 50 years of age, and is equally distributed between males and females. Its association with GBC remains a topic of debate in the literature (between 0 and 20%). Symptoms are non-specific and generally similar to those of acute or chronic cholecystitis. XGC, when associated with altered health status, leads to the suspicion of GBC. XGC can also come to light due to an acute complication of cholecystolithiasis, in particular, gallstone migration. Imaging by sonography and CT scan is suggestive, but magnetic resonance imaging is more specific. In difficult cases, biopsy may be necessary to eliminate the diagnosis of tumor. In case of pre- or intra-operative diagnostic doubt, the opinion of a hepatobiliary specialty center can be of help. When diagnosis of GBC has been eliminated, laparoscopic cholecystectomy is recommended, although with a high risk of conversion to laparotomy and complications.