We report an extremely rare case of dural arteriovenous fistula(DAVF)involving the superior sagittal sinus(SSS)that presented as a primary intraventricular hemorrhage(PIVH). A 79-year-old man who presented with disturbance of consciousness and vomiting was transferred to our hospital. The initial head CT revealed an intraventricular hemorrhage in the lateral, third, and fourth ventricles, predominantly involving the right lateral ventricle associated with obstructive hydrocephalus. CT angiography and three-dimensional rotational angiography(3D-RA)revealed SSS-DAVF with retrograde venous drainage from the cortical venous system to the subependymal venous system through engorged superficial/anastomotic/deep medullary or transcerebral veins, showing a pseudophlebitic pattern. A severe stenosis of the SSS was observed at the site just proximal to the shunting point. A varix of the right transvers caudate vein(TCV)was observed within the thickest portion of the hematoma in close contact with the wall of the right lateral ventricle. A stenosis of the branching point of the right TCV was detected on the 3D-RA image. The DAVF was completely cured after transarterial embolization. The varix gradually shrank and finally disappeared after 2 months. This is the first report of a case of PIVH due to SSS-DAVF with a subependymal varix considered as the bleeding point. Serial gadolinium-enhanced MR images clearly showed the shrinking of the varix. Our case is the second reported case of spontaneous resolution of the varix after embolization of the DAVF. The obstructive changes of the two venous outflow pathways(SSS/TCV)might further worsen the state of venous congestion in the deep medullary venous system and eventually lead to formation and rupture of the varix.

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