An 11-year-old girl presented with chronic cough and recurrent hemoptysis. On examination, she had features of right heart failure and cyanosis, with severe pulmonary hypertension on echocardiogram. Computed tomography pulmonary angiography showed aneurysmal dilatations of the pulmonary artery with elevated erythrocyte sedimentation rate and C-reactive protein and positive human leukocyte antigen B51. A diagnosis of Hughes-Stovin syndrome (vascular variant of Behcet's syndrome) was confirmed, and she was started on immunosuppression, on which there was improvement.