The authors report on the case of a 28-year-old woman presenting with an intraosseous arteriovenous fistula (AVF) located in the left parietal bone. The fistula was formed by direct arteriovenous shunts connecting branches of the left middle meningeal and superficial temporal arteries with a parietal diploic vein. Drainage occurred through both the external and internal jugular venous systems. Therapy consisted of combined surgical and endovascular approaches. The results of a pathological examination of the resected AVF showed mild enlargement of the diploic space. The angiographic appearance, pathological anatomy, and treatment of this rare lesion are discussed, as is a possible relationship between diploic AVFs and the development of aneurysm bone cysts.