Body overgrowth may be generalized or may affect certain areas. We present a seven-year-old African male with progressive, asymmetric, postnatal overgrowth of the left side of his face and left upper limb. The impression of proteus syndrome (PS) was made based on established clinical diagnostic criteria presented in the literature. Our objective is to highlight the diagnosis based on clinical features, investigations, and a multidisciplinary approach to the management of proteus syndrome.