This retrospective study summarized the technique aspects and effectiveness of transvenous balloon-assisted Onyx embolization treating selected dural arteriovenous fistulas of hypoglossal canal (HCDAVFs). Eight patients of HCDAVFs from January 2010 to December 2016 in a single institution were reviewed retrospectively. There were six males and two females aged from 30 to 69 years (mean age, 52.8 years). Eight patients presented with pulsatile tinnitus, four associated with ocular symptom, and one accompanied with tongue muscle atrophy. All lesions were with accessible venous approach from ipsilateral internal jugular vein. The microcatheter was positioned in the venous pouch from internal jugular vein; the remodeling balloon was advanced from internal jugular vein into inferior petrosal sinus. The balloon having 4 mm in diameter and 15 mm in length was inflated to temporarily block the antegrade venous drainage from fistulous pouch to internal jugular vein during the injection of Onyx. Approximately 1- to 2.1-ml Onyx-18 was used as the sole embolic material to obliterate the lesions. All lesions were occluded completely in a single-session embolization without procedural complications and postoperative new symptom. The follow-up period ranged from 6 to 13 months. Preoperative ocular symptom and tinnitus were resolved completely in all patients. The follow-up angiograms of three patients demonstrated durable occlusion. Our experience in this small series of patients indicated transvenous balloon-assisted Onyx embolization was a feasible and effective option for treating selected HCDAVFs.