Background Ulnar longitudinal deficiency (ULD) has been reported to be 1 in 100,000 live birthsCase presentation a 57-year-old healthy man with a fracture of the right medial malleolus was referred to our Orthopedic Center. Upon physical examination, a congenital abnormality in the patient's right upper extremity was observed. It was markedly shortened, pronated, and internally rotated. Radiological investigations revealed radiohumeral synostosis along with a complete absence of ulna and carpal bones, as well as shortened and bowed radius. Besides, only the thumb and the first metacarpal bone were present in the hand, and the wrist deviated towards the ulna. Taking into account the patient’s clinical findingsConclusion it is concluded that he likely falls under type D of Cole and Manske’s classification and type V of Goldfarb’s.