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Abstract Background Although brain arteriovenous malformations (AVM) usually remain asymptomatic during pregnancy, they can cause intracranial hemorrhage and lead to serious neurological deficits. Nowadays, it is accepted that treatment of a ruptured brain AVM during pregnancy should be based on neurologic, not obstetric, indications. Recently, endovascular treatment has been recognized as a treatment option associated in pregnant patients with brain AVMs. Case presentation A 34-year-old woman presented at 25 weeks of gestation with a history of severe headache followed by severe consciousness disturbance. Brain CT showed a subcortical hematoma in the right occipital lobe along with bilateral intraventricular hematomas. A cerebral angiogram was performed to confirm the diagnosis, which revealed right occipital AVM. At 27 weeks of gestation, endovascular embolization of the AVM was attempted under general anesthesia. The feeding artery and the nidus were simultaneously obliterated by injection of 50 % n-butyl-cyanoacrylate. As a result, the blood flow into the nidus was drastically decreased and the risk of re-bleeding was substantially reduced. At 38 weeks of gestation, elective cesarean section was performed to deliver the baby under combined spinal-epidural anesthesia (CSEA). An infant weighing 3665 g was delivered, with Apgar scores of 8 and 9 at 1 and 5 min, respectively. Postoperative analgesia was provided by a continuous infusion of ropivacaine via the epidural catheter. The infant was confirmed as not having any congenital anomalies. On POD 5, both of the patient and the infant were discharged home without any medical problems. The mother has shown no evidence of re-bleeding from the intracranial lesion since, and the infant is thriving well. Conclusions Endovascular treatment in pregnant women is associated with various unique concerns. However, it can be carried out safely and effectively and is useful not only for saving the mother’s life but also for allowing the pregnancy to continue to term.