Macrodystrophia lipomatosa (MDL) is a rare developmental anomaly causing localized gigantism. It is due to progressive proliferation of all mesenchymal elements, especially fibroadipose tissue. This developmental anomaly is more common in lower extremity but sometime also found in upper extremity. The disease is almost always unilateral, with an equal incidence in both sex. MDL is usually reposted and diagnosed during childhood. We report a case of macrodystrophia lipomatosa in a young adult which started from a finger and gradually affected the whole hand leading to amputation of hand from wrist joint. This is a case of late presentation. Involvement of whole hand, as seen in our case, is not yet reported in English literature to the best of our knowledge.