PTEN hamartoma tumor syndrome (PHTS), which comprises Cowden syndrome (CS; OMIM 158350), Bannayan–Riley–Ruvalcaba syndrome (BRRS; OMIM 153480), and Proteus-like syndrome, represents a spectrum of hamartomatous overgrowth manifestations associated with germline mutations in the PTEN gene2. The gene discussed is PTEN; the disease is Cowden disease.