Background. Arteriovenous malformations of the pancreas are very rare, most frequently congenital ones, but tending to increase frequency, predominantly due to the introduction and use of new diagnostic modalities. They usually present with gastrointestinal bleeding and abdominal pain. Case report. The autors presented a 52-year-old woman in whom, during the investigation for the abdominal pain and gastrointestinal bleeding, an ultrasonography showed a hypoechogenic lesion within the head of the pancreas 3 cm in diameter. Endoscopic ultrasound showed an irregular hypoechogenic lesion. Computed tomography showed a low density area within the lesion (< 10HU). At endoscopic retrograde cholangiopancreatography, a cystic lesion within the head of the pancreas was filled with contrast from the normal pancreatic duct. Angiography showed an arteriovenous malformation within the head of the pancreas. The patient underwent pylorus preserving cephalic duodenopancreatectomy. The diagnosis of arteriovenous malformation, probably of congenital origin, was confirmed on histology. Conclusion. Arteriovenous malformations should be considered in patients with gastrointestinal bleeding of unknown etiology. In this case, the lesion was taken into account preoperatively, based on color Doppler ultrasonography and angiography. The best results of treatment can be achieved with resection, but it has to be performed before an eventual portal hypertension takes place.