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Background: Ulnar club hand (ulnar deficiency) is a rare congenital disorder of the upper extremity. In the Flatt series among 2758 congenital disorders of upper extremity only 28 cases of ulnar deficiency were reported. Due to its rarity and variations in presentation current data in the management of the deformity is very limited. Here we present our experience and results in comparing management of ulnar deficiency.Methods: We include all of the ulnar club hand patients (five boys and one girl with seven involved extremities) from 1993 to 2006. After recognizing the type of deformity the classic management approach was performed that was splinting in corrective position until six months of age and then anlage resection. Syndactyly release was done in appropriate age according to involved rays, other operations for restoration of apposition was done after 18 months of age. Two of our patients were neglected, the first one was a 12 years old boy without any ulnar deviation but with syndactyly of the remaining rays and the other was a 32 years old male with severe ulnar deviation and partial syndactyly who is a skillful worker. We determined the effect of anlage resection on ulnar deviation of the wrist and restoration of opposition and syndactyly release on function of the limb.Results: In short term follow up, anlage resection was effective in prevention and correction of ulnar deviation, however the deformity was partially recurred later. Surprisingly, the function of the limb was not significantly affected with the extent of the deformity. On the other hand, the operations used for opposition of the thumb like first metacarpal rotational osteotomies and tendon transfers for powerful opposition, were more effective in the hand and also limb function compared with anlage resection alone. Conclusions: Due to our observation of the neglected cases, the most important factor in the function of the hand is the function of the thumb, thus we believe that restoration of opposition and syndactyly release may be more effective than anlage resection on limb function in Ulnar Club Hand patients