A 46-year-old man was operated on for cholelithiasis with chronic cholecystitis. On gross inspection of the resected gallbladder, a slight thickening in the body wall, in an area measuring about 1 x 0.5 cm, was noted. On light microscopic and immunohistochemical examinations, the lesion was diagnosed to be a rhabdoid tumor. After a thorough review of the literature we failed to find any reference to such a lesion in the gallbladder. This is the first known case report of a rhabdoid tumor of the gallbladder.