Dieulafoy's lesion is defined as a small mucosal defect overlying an abnormal, large caliber submucosal artery that protrudes through the gastrointestinal mucosa. This lesion is a rare cause of massive upper gastrointestinal bleeding in the pediatric population and extremely rare in neonates. We report a 1-day-old neonate who presented with massive gastrointestinal bleeding caused by a gastric Dieulafoy lesion, which was successfully treated by endoscopic hemoclipping without any complications.