Chondromyxoid fibroma is a rare benign cartilaginous tumor frequently involving the metaphysis of the long bones of the lower limbs of children and adolescents. We present a case of chondromyxoid fibroma involving the entire distal phalanx of a big toe causing macrodactyly, which was treated by excision and reconstruction using iliac bone graft instead of amputation. The patient was satisfied cosmetically and there was no evidence of recurrence four years after surgery.