Macrodystrophia lipomatosa is a rare form of non-hereditary congenital localized gigantism. Radiographs and magnetic resonance imaging of one such case in a 3-year-old girl is presented in which the entire right-lower limb was affected. Radiographs revealed excessive soft tissue hypertrophy and exostoses-like bony overgrowth from the metatarsals. Magnetic resonance images showed excess unencapsulated fatty proliferation and fatty infiltration of the involved muscles.