The authors report a case of an unusual site for chondromatosis in the hand: at the distal interphalangeal joint of the third ray. Swelling is the most important sign, without a history of trauma. A study of the literature revealed twelve publications of osteochondromatosis localised to the hand and only five at the interphalangeal joints of the fingers. The authors stress the necessity of a pathological examination and early surgery before joint destruction begins.