Macrodystrophia lipomatosa is a rare form of congenital localized gigantism, which is characterized by slowly progressive overgrowth of the mesenchymal elements, especially the fibroadipose tissue,in a limb. Frequently, multiple contiguous digits are involved on the lateral side of the hand or the medial side of the foot. Overabundance of the fibroadipose tissue and osseous overgrowth are most remarkable at the distal ends of the extremity. Macrodystrophia lipomatosa involving an entire limb has been previously reported for only one case in a 45-year-old adult male;1 however, no details of treatment were described. Here, we present the first report of successful limb salvage surgery of macrodystrophia lipomatosa in an adolescent involving an entire upper extremity.