Dieulafoy's lesion is a rare, but significant cause of gastrointestinal hemorrhage. This is an artery that despite its peripheral, submucosal course within the gastrointestinal wall, remains the same caliber, which makes it susceptible to damage. The majority of these lesions are located in the proximal part of the stomach, along the lesser curve, and this is due to the special vascular architecture of the area. Approximately only one third of lesions are extragastric and we can find them in the esophagus, and the small and large intestine. The typical clinical presentation is fierce: patients present with melena and hematemesis, frequently followed by hemodynamic instability which requires blood transfusions. Endoscopy is considered the gold standard in diagnosis as well as in therapy. Up to 30% of Dieulafoy’s lesions remain undetected because they are very small and inconspicuous. Endoscopy can then be repeated or other methods such as angiography, scintigraphy or endoscopic capsules are applied. In therapy the first step should be to stabilize a bleeding patient and only afterwards attend to the hemostasis. Methods available during endoscopy include local injection of epinephrine or sclerosing agents, thermocoagulation that includes electrocoagulation, argon plasma coagulation and laser photocoagulation, and finally, mechanical methods such as band ligation and hemoclip. There is no consensus that would distinguish a particular therapeutic method as the best, and for that reason the choice depends on the availability of equipment, experience, and physicians' skills. When these methods fail to achieve hemostasis, the last choice in the therapy is surgical.