Abstract: Objective To discuss the clinical characteristics, diagnosis, treatment and prognosis of moyamoya disease patients with posterior circulation lesions, so as to improve the understanding of this disease. Methods A total of 150 moyamoya disease patients with posterior circulation lesions who were diagnosed and treated in Department of Neurology, the Affiliated Hospital of Xuzhou Medical University from January 2013 to February 2019 were enrolled and their clinical data were retrospectively analyzed. Results In the current study, age distribution was within 10-14 year peak and 40-44 year peak, and there was no statistical difference between men and women in each age group (P>0.05). There were 14 patients (9.3%) with family history. The primary clinical characteristics were transient ischemic attack (TISA) in 70 cases (46.7%) and stroke in 38 cases (25.3%). All patients were diagnosed by DSA examination, with compensatory blood supply to various extent. All patients received symptomatic treatment after admission, where 134 patients underwent vascular revascularization, and were followed up for 24 months after discharge. There were no death cases and serious complications. Conclusions The epidemiological, clinical and imaging characteristics of moyamoya disease patients with posterior circulation lesions were analyzed, which is helpful for early diagnosis and treatment.