Maternal cortical vein thrombosis is a potentially fatal complication of pregnancy and the puerperium. Patients may present with focal neurologic deficits, seizures, or symptoms indicating increased intracranial pressure. Associated conditions include maternal dehydration and preeclampsia or frank eclampsia. Parturients may require anesthesia for various types of delivery. Safe administration of appropriate anesthesia must take into account the possible presence of a coagulopathy or reduced intracranial compliance. Case presentations, a literature review, possible pathogenetic mechanisms, and specific anesthetic considerations are discussed to enable the obstetric anesthesiologist to develop a rational plan of management.