Congenital lipomatosis of the face is a very rare syndrome defined as a collection of non-encapsulated mature lipocytes infiltrating local tissues: resulting in cranio-facial deformities. Due to the normal psychomotor development of the children, aesthetic appearance often remains the main concern. After a precise description of a case, a complete overview of the literature data was made. New clinicopathologic data concerning bone involvement will be presented and analyzed in the light of embryonic growth.