Ulnar dimelia is a rare congenital anomaly of the upper extremity. The condition presents with double ulnae forearm and polydactyly with complete absence of radius bone and thumb. Clinically it presents not only as functional deformity but also as cosmetic deformity. The case presented here is of a one-and-a-half-year-old male baby, born with right side ulnar dimelia with no other musculoskeletal defect. We believe that the case presented here deserves reporting not only because of the rarity

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... the disease but also due to its difficult management requiring multiple staged operations and henceforth consequent neglect on the part of parents. An attempt is also made to do a brief literature review. Keywords: Ulnar dimelia; polydactyly; ulna; radius; thumb