The Proteus syndrome is a recently described congenital harmatosis consisting of numerous clinical features of great variety. Mainly affected are the musculoskeletal system, primarily by hemihypertrophy, macrodactyly, exostoses and kyphoscoliosis, and the skin and the subcutaneous tissue, primarily by pigmented nevi and subcutaneous tumors. These findings are diagnostic features of Proteus syndrome. We report typical manifestations of Proteus syndrome in a 12-year-old boy with brief review of literature.