Cerebral venous sinus thrombosis (CVST) is a rare cerebrovascular condition that affects approximately 5 per 1 million people annually, and develops in 0.5% of all stroke patients. Herein we report a case involving a 31-year-old woman with CVST. She initially presented with a 2-month history of intermittent headaches at the nape of her neck with cervical pain. Other than these symptoms, she was in apparently good health and was a nonsmoker. She had no children and did not take contraceptives. She became comatose with unequal pupil size and CVST was diagnosed. An autopsy revealed CVST that extended from the confluence of the sinuses to the transverse sinuses and tip of the superior sagittal sinus, as well as a thrombus that obstructed the right internal jugular vein. A correct and early diagnosis of CVST combined with heparin-based therapy and/or interventional endovascular strategies may be of benefit by preventing intracerebral extension of jugular venous thrombosis and subsequent serious or even fatal neurological sequelae.