Macrodystrophia lipomatosa is a rare form of congenital localized gigantism. A forty-year-old male patient presented with complaints of swelling and deformity in the right toe and difficulty in wearing shoes. Conventional radiographic examination, ultrasonography, and computed tomography showed dorsal and medial deviations in the right toe, hypertrophy and degeneration in bone structure, increased density in the medullary bone, and a lipomatous mass with infiltration to the adjacent soft tissue muscles. Partial removal of the lipomatous tissues was performed. Microscopic examination of the specimen revealed fat deposits, ligamentous, and neural tissue elements in normal structure. The patient had no complaints and there was no change in the size of the lesion within a two-year follow-up period.