Pulmonary arteriovenous fistula are an uncommon disorder, and are most frequently congenital, usually then associated with hereditary hemorrhagic telangectasia (Rendu-Osler-Weber disease). We present, to our knowledge, the first case of a pulmonary arteriovenous fistula detected by gadolinium-enhanced pulmonary magnetic resonance angiography and confirmed by digital subtraction pulmonary angiography in a patient where the CT scan was unremarkable.