Proteus syndrome is an extremely rare genetic disorder characterized by an asymmetrical overgrowth of skin, bones, muscles, fatty tissues, and blood and lymphatic vessels. We present a case of a six-year-old boy with proteus syndrome who underwent bone scintigraphy for suspected osteomyelitis. Bone scintigraphy ruled out osteomyelitis and suggested cellulitis. In addition, it demonstrated striking characteristic deformities, which need to be emphasized. Knowledge of these findings will avoid misinterpretation of bone scintigraphy in patients with proteus syndrome.