The pathogenesis of cerebral arteriovenous malformations (cAVMs) is still not well understood. Generally, cAVMs are thought to be congenital lesions originating prenatally. We report a 7-year-old boy diagnosed with a de novo cAVM after 3 years of recurrent epileptic seizures. MR imaging at 4 years of age was normal. Follow-up MR imaging 3 years later demonstrated a de novo 2-cm cAVM in the right occipital lobe, confirmed by conventional angiography. We reviewed five previously reported cases of de novo cAVMs who did not have a previous neurovascular abnormality. Including our case, recurrent epileptic seizures are the major presentation (83.3 %) before de novo cAVM occurrence. We suggest that epileptic seizure is a potential trigger of de novo cAVMs.