Case Description- A 3-month-old, female mixed breed dog was presented to the Veterinary Teaching Hospital of Ferdowsi University with an obvious deformity in its right forelimb. Clinical Findings- - In clinical examination, there was a separation from elbow joint to the end of digits and a long skin cleft between these separated regions was seen. Radiographic examination revealed a wide space between the radius and the ulna with a separated carpal joint. The limb had five digits which three of them were at the ulnar side and the others were at radial side. The characteristic proximal and distal carpal bone rows and distal radioulnar joint were absent. Treatment and Outcome- According to the clinical and radiographic findings the ectrodactyly was diagnosed, but upon to the owner' disagreement, the amputation was not done. Clinical Relevance- Ectrodactyly is a rare anomaly which can usually be treated surgically. In extremely severe cases such as the case described, amputation of the affected limb should be done.