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Radio-ulnar synostosis constitutes one of the rare and uncommon anomalies of the upper limb. It may be congenital where there is failure of segmentation of the radius and the ulna or acquired resulting in post-traumatic synostosis. Males and females are equally affected in congenital variety and bilateral involvement is found in half of those affected. The condition may be associated with other developmental anomalies or as a part of an underlying syndrome. Clinical presentations include subtle functional limitations of the elbow flexion and extension and pain is a rare complaint. However, a careful medical history, aided by physical examination and diagnostic tool can confirm the diagnosis. Here, we are reporting a case of proximal radio-ulnar synostosis with an aim to make clinicians aware of the possible differential diagnosis and thereby successful treatment outcome.