Syndromes and corresponding genes include: SMAD4 (DPC4) and BMPR1A for juvenile polyposis (JPS), STK11 for Peutz–Jeghers (PJS) and PTEN and AKT1 for PTEN hamartomatous syndromes (PHS) [1,2]. The gene discussed is SMAD4; the disease is Peutz-Jeghers syndrome.