Disorders of the central nervous system (CNS) in pregnancy continue to be a common reason for morbidity and mortality in mothers. Common CNS diseases during pregnancy and their anesthetic management are described in our chapter. Some disorders of central nervous system preexist pregnancy, like epilepsy, Parkinson disease, multiple sclerosis, intracranial lesions, benign intracranial hypertension, or migraine. Others may have an increased incidence in pregnancy, for example cerebrovascular disorders, including hemorrhagic or ischemic strokes. Experience in treating these CNS diseases is limited, thus management is largely based on isolated case reports, case series, and some review articles. A high suspicion for uncommon CNS conditions is vital since timely and intense treatment significantly affects the outcome. Important aspects of consideration for the care of the parturient with a CNS disorder are the pathophysiology of the lesion, effects of pregnancy on the lesion, and effects of lesion on pregnancy. Other important considerations in management are the effect of medical management, monitoring, and surgery on fetus, maternal versus fetal priority regarding surgical plan, timing, and route of delivery. Most importantly communication and coordination among the patient, her family, and the medical team (neurologist, neurosurgeon, obstetrician, anesthesiologist, medical consultants, and nursing staff) is paramount in the optimal management of mother and fetus.