Background Brain arteriovenous malformation (bAVM)-related epilepsy can significantly affect patient quality of life. We aimed to identify the factors associated with seizures occurrence and evaluate the long-term outcome following Onyx embolization in bAVM patients. Methods Between July 2014 and July 2016, 239 consecutive patients underwent treatment for bAVMs in our institute and were respectively analyzed. Demographics, seizure status and bAVM morphologic characteristics were recorded. Modified Engel classification was used to evaluate the long-term seizure outcomes. Results Of 239 bAVM patients, 68 (28.5%) initially presented with seizures. Seizure occurrence was associated with cerebral hemorrhage history, frontal-temporal location and arterial borderzone location. Of the 37 patients who presented with initial seizures and were treated with Onyx embolization, 23 (62.2%) were treated with antiepileptic drugs (AEDs) before Onyx embolization. At the last follow-up visit, 19 (51.4%) of the 37 patients reached modified Engel class I outcome. Of the 23 patients who had ever been treated with AEDs, 12 (52.2%) were still taking AEDs at the last follow-up visit. Single-factor analysis showed that arterial borderzone location was significantly correlated with higher modified Engel class outcome ( p = 0.046). Conclusion Patients with bAVM hemorrhage history, frontal-temporal location and arterial borderzone location were associated with seizure occurrence. Seizure-free status was not obtained in AVM patients with arterial borderzone after embolization, though it may have benefits in other ways. The seizure-free mechanism of bAVM with Onyx embolization is worth further study.