PTEN hamartoma tumor syndrome (PHTS), which comprises CS, adult Lhermitte-Duclos disease (LDD), Bannayan-Riley-Ruvalcaba syndrome (BRRS), and Proteus-like syndrome, represents a spectrum of hamartomatous overgrowth manifestations associated with germline mutations in the PTEN gene [4]. This evidence concerns the gene PTEN and Bannayan-Riley-Ruvalcaba syndrome.