Shaheen Syndrome, also known as hypohidrosis-enamel hypoplasia-palmoplantar keratoderma-intellectual disability syndrome, is related to pachyonychia congenita 3. An important gene associated with Shaheen Syndrome is COG6 (Component Of Oligomeric Golgi Complex 6). Affiliated tissues include skin and prostate, and related phenotypes are intellectual disability and recurrent respiratory infections