Cerebral venous thrombosis is an uncommon cause of stroke but remains a challenge for physicians faced with this diagnosis largely due to the variability in presentation. Anticoagulation, typically with intravenous heparin, remains the mainstay of treatment for stable patients and is sufficient in the majority of cases. However, a significant mortality rate exists for cerebral venous thrombosis due to patients who deteriorate or do not adequately respond to initial treatments. It is in these patients that more aggressive interventions must be undertaken. The neurosurgeon is often called on, either acutely for initial evaluation of the stroke or venous hemorrhage or after the failure of initial therapy for clot evacuation, hemicraniectomy, or thrombectomy. A proper workup must include a search for an underlying, correctable cause as well as thorough follow-up with correction of identified risk factors to decrease the risk of recurrent disease.