Congenital distal tibiofibular synostosis is a very rare anomaly. To date, only few case report of this entity has been reported. We report a new case and review the previously reported cases. Most of the previously reported cases are acquired and reported to be post-traumatic or iatrogenic in origin. We present a case of a 25-year-old man had neither a history of trauma nor infection around the lower leg and ankle, presented with progressively increasing deformity and vague discomfort in the right lower leg. Imaging revealed the typical features of synostosis unusually involving the distal tibia and fibula.