A case of Peutz-Jeghers Syndrome (PJS) presenting in infancy with gastric-outlet obstruction is described. PJS may become symptomatic at any age and should be suspected when there are obstructive symptoms or gastrointestinal blood loss in a baby with a positive family history. Contrast studies and endoscopy are useful in diagnosis and surveillance. Treatment requires a combination of endoscopy and laparotomy/laparoscopy. Because of the reported cancer risk, we recommend surveillance from the age of diagnosis.