Hereditary hemorrhagic telangiectasia ( HHT), or Rendu-Osler-Weber syndrome ( ROWS) is a very rare hereditary disease. The diagnosis is based on the clinical findings such as recurrent epistaxis, telangiectases, visceral arteriovenous malformations (AVMs) and family history. AVMs are found in the liver, lung or brain and could mimick the masses of these organs. Radiologic evaluation plays a critical role during diagnostic and therapeutic management of ROWS. Hence, radiologists should be aware of the diagnosis of HHT in the patients with AVMs, history of epistaxis and family history. We report a patient with multiple pulmonary AVMs secondary to HHT who has referred to our interventional radiology department for computed tomography guided transthorasic lung biopsy procedure with suspicious of malignancy.