RESUMO
Macrodystrophia lipomatosa is a rare congenital form of localized gigantism, characterized by an increase in all mesenchymal elements, particularly fibroadipose tissue. The areas of predilection are segments supplied by the median or plantar nerves. We report such a rare case in a thirty four year old male patient, who presented with a swelling of the right upper limb and a marked increase in the size of the right thumb, index finger and radial half of the right hand, present since birth, with progressive increase to the present size. Amputation of the right thumb and debulking of the palm was done. The specimen measured 26 x 18 x 12 cms. and the thumb alone measured 13 x 7 x 7 cm. histology revealed hypertrophy of adipose tissue in the subcutaneous compartment and infiltration into the nerve sheaths and muscles.