Cerebral venous sinus thrombosis (CVST) is a rare neurological emergency associated with significant morbidity and mortality. CVST has been found to account for up to 57% of pregnancy-related strokes. Pregnancy and the postpartum period are associated with physiological changes that lead to a hypercoagulable state, increasing the risk for CVST. The pathophysiological mechanism for CVST involves increased venule and capillary pressure as well as intracranial pressure, which lead to clinical manifestations such as headaches, seizures, and focal neurological deficits. Diagnosis involves imaging including computed tomography, magnetic resonance imaging, venography, and in some cases, cerebral angiography. Treatment options during pregnancy and postpartum include anticoagulation with low molecular weight heparin and symptomatic management. Further research is needed to elucidate the safety and efficacy of endovascular treatment techniques in severe or refractory cases of CVST, particularly during pregnancy and puerperium. In this review, we describe the physiological changes of pregnancy, outline the pathophysiological mechanisms and clinical findings of CVST, and discuss treatment approaches specific to pregnancy and the postpartum period.