Digital extensor muscle aplasia-polyneuropathy is a rare, hereditary motor and sensory neuropathy characterized by flexion deformities of the thumb and fingers, sensory deficit in the hand and polyneuropathic electrophysiologic findings in the limbs. Operation on the hands reveals extensor muscles and their tendons to be absent or hypoplastic. There have been no further descriptions in the literature since 1986. The disease is Monarch Disease Ontology term MONDO_0008809 (polyneuropathy-hand defect syndrome). Also known as: Hamanishi Ueba Tsuji syndrome, Hamanishi-Ueba-Tsuji syndrome.