IntroductionHughes-Stovin syndrome is a life-threatening disorder of unknown etiology. This condition is characterized by vasculitis, deep venous thrombosis and aneurysms that mainly involve the pulmonary arteries resulting in hemoptysis. It has been described in literature less than 40 times. However, we believe it is not very uncommon as it might be diagnosed as pulmonary embolism solely. In such cases, anticoagulation therapy augments the risk of life-threatening hemoptysis.Materials and methodsWe report the case of a 35 years old, Egyptian female patient with Hughes-Stovin syndrome, who initially presented with lower limb deep vein thrombosis and coughing of blood. Anticoagulation regimen for pulmonary embolism was given. This resulted in massive hemoptysis that was successfully controlled by medical therapy.ConclusionAdults who present with venous thrombosis and hemoptoic cough, with no predisposing factors of thrombosis, normal platelet count and coagulation, the possibility of Hughes-Stovin syndrome has to be considered.