Moyamoya disease (MMD) and dural arteriovenous fistula (DAVF) are two distinct types of intracranial lesion that share different pathogenic mechanisms. Under rare circumstances, patients with MMD have been reported to have concurrent DAVF. The present case study reports on a 47-year-old male admitted due to sudden headache. Head CT revealed hemorrhage of the right thalamus with ventricular extension. On CT angiography, the normal vasculature in the anterior and posterior circulation disappeared and was replaced by moyamoya-like vessels. The patient received conservative management and was discharged 3 days later. After three months, the patient was readmitted for acute cerebellar hemisphere infarction. Angiogram indicated that the DAVF (Cognard classification Ⅰ) was supplied by the left middle meningeal artery, occipital artery and posterior meningeal artery and drained into the transverse-sigmoid sinus and occipital sinus. Conservative management of the DAVF was adopted. The patient was stable and lived independently during a 4-year follow-up. A literature review of the reported cases was also performed to further characterize this rare entity. The management of DAVF concurrent with MMD depends on its clinical presentation and invasiveness. For patients with symptoms or cortical venous drainage, endovascular intervention should be performed. For asymptomatic DAVFs or those without cerebral venous drainage, close follow-up is a reasonable option.