We present a case of developmental venous anomaly associated with arteriovenous fistula supplied by a single arterial feeder adjacent to a large acute intracerebral hemorrhage. The arteriovenous fistula was successfully obliterated by superselective embolization while completely preserving the developmental venous anomaly. Two similar cases, including superselective angiographic findings, have been reported in the literature; however, we describe herein superselective angiographic findings in more detail and demonstrate the arteriovenous shunt more clearly than the previous reports. In addition, a literature review was performed to discuss the association of a developmental venous anomaly with vascular lesions.