Background: Cerebral arteriovenous malformations (AVMs) have been long thought to be a congenital anomaly of vasculogenesis in which arteries and veins form direct communications forming a vascular nidus without an intervening capillary bed or neural tissue. Scattered case reports have described that AVMs may form de novo suggesting that they can become an acquired lesion. Case report: Our case, a 32-year-old, right-handed, male presented with new onset seizure with history of previous episode of intracerebral hematoma which was confirmed by CT scan of brain for which surgical evacuation was performed 8 years back. Post-operative recovery was uneventful and no episode of seizure was noticed at that time. Five years later, he developed new onset of seizure which was partially controlled by conventional Antiepileptic drugs. Conclusion: This case was initially a case of congenital small cerebral AVM that bleeds and subsequently developed new onset of seizure which became intractable. It indicates that cerebral AVM is a congenital vascular lesion.