A case of essential carpal osteolysis is reported. The morphological appearance of the hands is described. They showed ulnar deviation; they were short and pudgy, the carpus and metacarpus being almost absent. The fingers were normal, but the left thumb was atrophic. Radiographies of the left hand showed an almost complete disappearance of the carpus and severe irregularities of the ulna and radius. Aetiology, treatment and prognosis are briefly discussed.