Introduction Moyamoya disease (MMD) is a progressive cerebrovascular disorder characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis with fine tortuous collateral vessels. In patients with MMD, systemic arteries, especially renal arteries, may also be affected. However, the pathogenesis of extracranial involvement in patients with MMD still is not fully understood. Herein, we report a female adolescent with Raynaud’s disease (RD) who later developed pulmonary hypertension and MMD. Case A 12-year-old girl was referred with a history of cold hands for 1 year. She showed painless purple discoloration and mottling of the skin in the hands during the cold exposure. To reduce the frequency and severity of Raynaud’s attacks, she received calcium-channel blocker (nifedipine) during the winter months. At 16 years of age, syncope and sudden onset of right anterior chest pain developed. The chest X-ray revealed bulging of the pulmonary trunk. The electrocardiogram showed right ventricular hypertrophy. The interventricular septal flattening, suggesting pulmonary hypertension, was found by echocardiography. Catheterization of the right heart showed that the systolic, diastolic, and mean pressures in the main pulmonary artery were 68, 27, and 47 mmHg, respectively. Selective pulmonary angiography demonstrated bilateral multiple stenosis or occlusion of the distal branch pulmonary arteries with a suspicious “string of beads” appearance. Because of a syncopal episode and 1-year history of intermittent right lower extremity weakness magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) of the brain were performed under the impression of systemic vasculopathy. Brain MRI/MRA revealed bilateral narrowing or occlusion of internal carotid arteries (ICA) and middle cerebral arteries (MCA) without definite cerebral infarction. The above findings with the presence of typical moyamoya vessels were also confirmed by means of cerebral angiography, and then the diagnosis of MMD was made. Abdominal aortogram showed normal renal arteries. She has been free of neurological symptoms after revascularization surgery. Conclusion The present case showing the association of MMD with pulmonary hypertension and RD provides further evidence for a better understanding of the pathogenesis of MMD as a systemic vasculopathy.