Papilledema is defined as optic disk edema that is secondary to increased intracranial pressure. During pregnancy, papilledema poses additional diagnostic and therapeutic challenges. As in the nonpregnant patient, the primary goal is to urgently determine the cause of the papilledema followed by implementing appropriate management for life-threatening conditions in a timely fashion while safeguarding the fetus. Papilledema may occur also in conditions that are not life threatening; in either case, papilledema may cause visual failure. We describe the two most common causes of papilledema during pregnancy, idiopathic intracranial hypertension and cerebral venous thrombosis. In the former, there is no threat to life, while in the latter, depending on the extent of the cerebral venous thrombosis, life-threatening medical issues may dominate the picture. In these conditions, attention to the prevention of visual failure is of major importance; however, treatment options may need to be modified to safeguard the developing fetus. In this article, we review the current diagnostic and treatment options for patients with papilledema, emphasizing special considerations for the pregnant patient, including a chart to help the clinician differentiate between the different conditions causing papilledema. A flow chart suggests an approach as to how to monitor vision function and steps to take to prevent visual loss in these conditions causing papilledema. Drugs that may be considered in the management of papilledema are reviewed, and the FDA information regarding their safety for the fetus is provided.