Dieulafoy's lesion is a rare arterial malformation that can cause massive gastrointestinal hemorrhage. The lesion occurs most commonly in the proximal stomach. The esophagus is not a common location for this lesion. We present the case of a 25-year-old woman who was admitted to our emergency unit with the findings of hematemesis and melena. Early upper gastrointestinal endoscopic examination revealed a Dieulafoy's lesion, which was located in the distal esophagus. Endoscopic band ligation stopped the bleeding successfully. The patient was discharged 3 days after the band ligation without any complications. Dieulafoy's lesion may cause severe, life-threatening bleeding. Endoscopic diagnosis can be difficult because of the small size and obscure location of the lesion. An abnormally dilated artery that penetrates through the mucosa constitutes the etiology. Endoscopy plays an important role in the diagnosis and treatment of this pathology. Despite widespread awareness of this entity, it may present a real challenge for the endoscopist due to the small size and hidden location of the lesion. The endoscopic approach to occult gastrointestinal bleeding for the diagnosis of vascular malformations is accepted as a quick and safe diagnostic method.