SUMMARY: Introduction: The Dieulafoys lesion is an infrequent but important cause of gastrointestinal haemorrhage. These lesions have been described at the gastric level and less frequently in the duodenum, jejune and colon. Macroscopically it is an arterial malformation, with tortuosity and elongation of the vessels that appear like a small defect in the mucosa and by histology as long vessels that surround the sub mucosa below the mucosa. It has an incidence of 0,3-1,5% as cause of massive gastrointestinal bleeding. Clinical case: 4 years old male that presents with a foreign body (corn seed) in the airway that required bronchoscophy. During admission presents frequent melena with anemia and hemodynamic instability. Rectosigmoidoscopy showed lower gastrointestinal bleeding without abnormal findings. Gammagram reported hypercaptant zone at the splenic angle of the colon. During surgery, trans-operating colonoscopy demonstrated hemorrhagic punctiform lesions with blood effusion at the splenic angle of the colon, and a segment of the colon was resected. Histopathology reported congestive blood vessels with proliferation of medium-caliber vessels, surrounded by fibrous stroma with linfohistiocitic infiltrate in the serosa, confirming the diagnose of Dieulafoys lesion. Discussion: This is an infrequent cause of gastrointestinal bleeding that is difficult to diagnose when it presents in an inusual location. Gammagram is helpful for the diagnosis. If the gastrointestinal bleeding compromises the life of the patient, definite treatment should be surgical. Arch Venez Pueric Pediatr 71 (1): 23 - 26