Aim To investigate the clinical and imaging characteristics of moyamoya disease complicated with coronary artery stenosis. Methods The clinical manifestations, imaging characteristics and prognosis of 8 patients with moyamoya disease complicated with coronary artery stenosis were analyzed. Results 8 cases of moyamoya disease were acute onset, including 6 cases of cerebral infarction and 2 cases of cerebral hemorrhage. Among them, 3 cases underwent coronary artery stent implantation. 8 cases were improved and discharged, and followed up for a period of 3 months, 1 case died of recurrent cerebral hemorrhage, and the other patients had different degrees of sequelae. Conclusion Moyamoya disease combined with coronary artery stenosis is extremely rare, and vascular lesions of moyamoya disease may not only involve the cerebral vessels.