Cardiofaciocutaneous syndrome is a rare genetic disorder. It is characterized by craniofacial dysmorphism, congenital heart disease, ectodermal abnormalities, developmental delay, and central nervous system disorders. We discuss the case of an 11-year-old boy with cardiofaciocutaneous syndrome presenting with ulerythema ophryogenes and phenotypic features similar to Noonan syndrome.