A new disorder describing multiple hamartomas distinct from neurofibromatosis and Klippel-Trenaunay-Weber syndrome was first reported in 1979. It was named Proteus syndrome after the Greek god Proteus, the polymorphous, who could change his shape at will to avoid capture. The clinical manifestations are extensive, including cranial exostoses; progressive enlargement, asymmetry and disfigurement of the skull; macrocephaly; exostoses of the ear canals, nasal bridge, and alveolar ridge; partial gigantism of the hands or feet, asymmetry of the limbs, plantar hyperplasia, hemangiomas, lipomas, lymphangiomas, varicosities, verrucous epidermal nevi, and long bone overgrowth. A case report of Proteus syndrome is presented and discussed along with a review of the pertinent literature.