Hughes-Stovin syndrome is a scarce entity associating pulmonary artery aneurysms and deep venous thrombosis. It was considered to be a form of Behcet’s syndrome at its initial feature. We present a case of a 27-year-old man having a Hughes-Stovin syndrome. The patient had a history of left ilio-femoral thrombosis and suffered from recurrent oral ulcers. He presented with recurrent hemoptysis. A giant pulmonary aneurysm was confirmed by chest CT scan. A right femoral thrombosis was shown on the inferior legs doppler ultrasonography. The patient was treated by steroids and cyclophosphamide and proposed to an embolization to prevent recurrence of symptoms.