Cerebral venous sinus thrombosis is an uncommon disease marked by clotting of blood in cerebral venous, or dural sinuses, and, in rare cases, cortical veins. It is a rare but potentially fatal cause of acute neurological deterioration previously related to otomastoid, orbit, and central face cutaneous infections. After the advent of antibiotics, it is more often related to neoplasm, pregnancy, puerperium, systemic diseases, dehydration, intracranial tumors, oral contraceptives, and coagulopathies are the most common causes, but in 30% of cases no underlying etiology can be identified. It has been found in association with fibrous thyroiditis, jugular thrombosis after catheterization, or idiopathic jugular vein stenosis. Other factors include surgery, head trauma, arterio-venous malformations, infection, paraneoplastic, and autoimmune disease. This article presents a comprehensive review of cerebral venous sinus thrombosis etiologies.