Cerebral venous thrombosis (CVT) presenting as subarachnoid hemorrhage (SAH) is infrequent. We present the case of a man with CVT of the right transverse sinus who presented with a SAH in the right parietal sinus. In this case, we describe a hyper-homocysteinemia in a heterozygous patient for the methylenetetrahydrofolate reductase C667T mutation. Our report highlights the value of an early diagnosis of CVT, the importance of identifying possible causes that could be reversed with an appropriate treatment, and the controversy about the timing for starting anticoagulation therapy in such cases.