A 35 year old male presented with pain in right middle finger since 5 y associated with shortening of right middle finger since 3 y. He had dull aching pain and blackish discoloration of the finger since 5 y. Examination revealed painful, restricted movements at inter-phalangeal joint and shortening of the finger of about 4 cm. After a thorough work up of the patient, he was diagnosed to have Gorham’s disease of the third metacarpal which was confirmed by histopathology reports. The patient was managed by surgical reconstruction to provide a functional and cosmetically acceptable hand.