This study investigated the incidences of persistent primitive arteries in patients with moyamoya disease, unilateral moyamoya disease, and quasi-moyamoya disease. Cerebral angiograms of 50 patients (39 moyamoya disease patients, 6 unilateral moyamoya disease patients, and 5 quasimoyamoya disease patients) were retrospectively reviewed. There were 35 females and 15 males, aged from 3 to 63 years (mean 27.4 years). Persistent primitive carotid-basilar artery anastomoses were observed in three patients: primitive hypoglossal artery in one moyamoya disease patient, primitive trigeminal artery variant in one unilateral moyamoya disease patient, and an anastomosis between the accessory meningeal artery and the anterosuperior cerebellar artery in one quasi-moyamoya disease patient. The ophthalmic artery originated from the middle meningeal artery in three moyamoya and two quasi-moyamoya disease patients. The incidence of the persistent primitive arteries is significantly higher in patients with moyamoya disease (10.7%) and quasi-moyamoya disease (60%) than in patients with other disease (0.67%) (p < 0.001), so congenital factors may be important in the pathogenesis of moyamoya disease.