We report the case of a 4-year old boy presenting with a polymalformative syndrome made of cutaneous and subcutaneous lesions associated with visceral and skeletal lesions. The cutaneous lesions consisted of 3 verrucous hamartomas, one haemangioma and one lymphangioma. The subcutaneous lesions were melting of panniculus adiposus, plantar tumefaction and diffuse lipomatosis of the peritoneal and retroperitoneal cavities. The skeletal lesions were uneven lower limbs and dorsal scoliosis. Atrophy of the intestinal villi was also found. The diagnosis of Proteus syndrome was made.