Filum terminale arteriovenous fistulae are a rare type of arteriovenous shunt generally characterized by a single direct communication between the artery of the filum terminale and a single draining vein. These intradural arteriovenous shunts are three times more common in men than women (mean age 55 years). Symptoms are related to venous congestion, vascular hypertension, and a putative chronic steal phenomenon which result in spinal cord ischemia and myelopathy. Interestingly, hemorrhage has never been reported as a mode of presentation. MRI demonstrates increased flow voids and T2 changes involving the conus and the lower spinal cord, and these findings are not dissimilar from those seen with the more common type 1 spinal dural arteriovenous fistulae. Thus conventional spinal angiography is necessary for a definitive diagnosis and to localize exactly the site of the fistula. Both surgical interruption of the fistula and endovascular embolization are safe and effective therapeutic modalities. However, because of the very small caliber of the feeding artery, endovascular therapy is often not feasible; and thus, surgery remains the method more commonly utilized for their treatment. Definitive treatment consists of obliteration of the direct arteriovenous shunt. In this review, we describe the anatomy, pathophysiology, clinical presentation, imaging, and treatment options of these less common intradural arteriovenous shunts.