Bannayan-Riley-Ruvalcaba syndrome (BRRS) is a rare congenital disorder characterized by hamartomatous intestinal polyposis, lipomas, macrocephaly and genital lentiginosis. The disease is Bannayan-Riley-Ruvalcaba syndrome (Monarch Disease Ontology term MONDO_0007924). Also known as: BRRS, Bannayan syndrome, Bannayan-Zonana syndrome, Myhre-Riley-Smith syndrome, RILEY-SMITH syndrome, Ruvalcaba-MYHRE-SMITH syndrome, macrocephaly with multiple lipomas and hemangiomas.