Background:Ruptured venous aneurysm is often seen with arterio-venous malformation (AVM) or developmental venous anomaly (DVA). However, isolated venous aneurysm is unusual.Case Description:We present a case of ruptured venous aneurysm that presented with subarachnoid hemorrhage (SAH) and intraventricular hemorrhage (IVH). Digital substraction angiography (DSA) revealed a saccular contrast filling pouch in the left lateral aspect of cervicomedullary junction (CMJ). Endovascular intervention was not a viable option. During surgery, a saccular pliable structure approx. 1.5 × 1 cm was found in the subarachnoid space that was clipped and excised. There were no arterial feeders, no evidence of surrounding AVM, and no dilated perimedullary vein.Conclusion:This is perhaps the first reported case of ruptured venous aneurysm (without associated AVM) of CMJ, which was successfully managed surgically. The possible etiologies remain an unnoticed head trauma or a congenital vessel wall abnormality. Surgically clipping and excision remains the treatment of choice for such lesion.