We present two sporadic cases of complete ulnar hemimelia, a rare congenital defect. In one case, ulnar hemimelia was associated with tridactyly and elbow malrotation with radiohumeral synostosis; in the second case, ulnar hemimelia was assciated with mono-metacarpal bidactyly and anterior cubital webbing of the elbow with fixed flexion. To the best of our knowledge, there is dearth of information on these combinations of anomalies in the literature and their management remains a challenge.