Osler-Rendu-Weber Syndrome or hereditary hemorrhagic telangiectasia (HHT) is characterized by telangiectasia of the skin and of the mucous membranes and intermittent bleeding from vascular abnormalities; in about 20% of the patients pulmonary arteriovenous malformation is present. Pulmonary arteriovenous malformation is a congenital anomaly in the lung which causes shunting of venous blood in the pulmonary artery to systemic circulation, resulting in cyanosis, polycythemia and clubbing. Recently we experienced a case of multiple pulmonary arteriovenous malformation associated with the telangiectatic change of the cerebral artery in a 16-year-old male patient, which was confirmed by pulmonary angiography.