Macrodystrophia lipomatosa with ulnar distribution in hand

Macrodystrophia lipomatosa is a rare, non-hereditary congenital anomaly characterized by localised gigantism in the form of macrodactyly affecting a part of limb and rarely entire limb due to overgrowth of all mesenchymal elements. Radiological and pathological hallmark is the disproportionate fibroadipose tissue proliferation in subcutaneous tissue, nerve sheaths, and periosteum that lead to soft tissue and bony enlargement. We present the case of a twenty years old female who presented with history of gradual enlargement of the medial aspect of right hand along ulnar nerve distribution since birth. MRI showed hypertrophy of bones and soft tissue with fatty proliferation, leading to the diagnosis of macrodystrophia lipomatosa.