Xanthogranulomatous cholecystitis (XGC) is an uncommon, focal or diffuse destructive inflammatory disease of the gallbladder that is assumed to be a variant of conventional chronic cholecystitis. A 36-year-old male was admitted to Chonnam National University Hospital with a 10-day history of right upper quadrant pain with fever. 15 years ago, he was first diagnosed as having hemophilia A, and has been followed up in the department of Hematology. Computed tomogram (CT) revealed a well-marginated, uniform, marked wall thickening of the gallbladder with multiseptate enhancement. Magnetic resonance imaging (MRI) demonstrated diffuse wall thickening of the gallbladder by viewing high signal foci with signal void lesions. After factor VIII replacement, exploration was done. On operation, the gallbladder wall was thickened and the serosa were surrounded by dense fibrous adhesions which were often extensive and attached to the adjacent hepatic parenchyma. There was a small-sized abscess in the gallbladder wall near the cystic duct. Dissection between the gallbladder serosa and hepatic parenchyma was difficult. Cross sections through the wall revealed multiple yellow-colored, nodule-like lesions ranging from 0.5-2 cm. There were also multiple black pigmented gallstones ranging from 0.5-1 cm. The pathologic findings showed the collection of foamy histiocytes containing abundant lipid in the cytoplasm and admixed lymphoid cells. Histologically, it was confirmed as XGC. We report a case with XGC mimicking gallbladder cancer in a hemophilia patient.