Arteriovenous fistula (AVF) occurring within the craniocervical junction (CCJ) is an uncommon vascular anomaly. A 56-year-old man presented with cervical pain and gait disturbances. Computed tomography revealed a hematoma in the epidural space at the cervical spinal level. Subsequent cerebral angiography revealed an epidural shunt at the CCJ, leading to a diagnosis of epidural AVF (EDAVF). Transvenous embolization, performed under general anesthesia, successfully resolved the shunt. Transarterial embolization of CCJ-EDAVFs carries a substantial risk of ischemic complications. Conversely, transvenous embolization poses a lower risk of ischemic complications and has potential as a beneficial treatment option for CCJ-EDAVFs. Herein, we present a unique case of CCJ-EDAVF that was successfully treated with transvenous embolization.