Macrodactyly is a rare congenital malformation characterized by an increase in the size of all the elements or structures of a digit or digits. The phalanges, tendons, vessels, subcutaneous fat, finger nails, and skin are all enlarged, but the metacarpals are not affected. The condition is most frequently found in the index and long fingers, and its etiology remains unexplained. Macrodactylism associated with neurofibroma of the median nerve in the left middle finger, in a 13 year old girl, is presented with a review of the literature, and diagnosis was confirmed by pathological examination. Chief complaints were enlargement of the left middle finger and a soft tissue mass in the left palm since birth. There was no change in motor and sensory function of the hand. X-ray showed enlargement of phalanges and a diffusely enlarged soft tissue shadow in the middle finger. We performed epiphysiodesis of the proximal, middle and distal phalanges of the left middle finger with exploration of the left median nerve to the wrist joint. An enlarged fibromatous median nerve, left side, was found with very large abundant fat lobules around it.