Introduction Acrorenal syndrome is autosomal recessive inherited disorder commonly associated with congenital renal disorders and ipsilateral hand/foot anomalies. The hand and foot deformities corrections are challenging to achieve a good functional and cosmetic result. We described a case of acrorenal syndrome with suppressed radial elements and absent thumb in whom we were able to reconstruct the hand. Case Report A 5-year-old girl presented to us with right renal agenesis and right hand/foot deformity. The hand showed a cleft hand with central deficiency, index finger hypoplastic, and syndactylyzed to middle finger, absent thumb. The hand was nonfunctional because of absent thumb. To improve the functions, it was decided to proceed with thumb reconstruction. The middle finger was pollicized to regain tripod grip and thereby the functions was enhanced. Discussion The cleft hand belongs to “failure of finger ray induction group” in classification by “International Federation of Societies for Surgery of the Hand (IFSSH).” Absence of thumb and first web space makes it a strong indication for surgical reconstruction. In our case, thumb was addressed by pollicization of middle finger and we were able to provide a good tripod grip.