Pseudotumor cerebri, a disorder of intracranial pressure regulation, is characterized by elevated cerebrospinal fluid pressures. The symptoms and signs are those of elevated intracranial pressure alone. Extensive endocrine studies in patients with the primary disease have generally been normal. The major threat to the patient with pseudotumor cerebri is loss of vision; severe deficits occur in 4% to 12% of patients. The causative mechanism is not known. There are no controlled studies of treatment and interpretation of uncontrolled studies is limited by the high rate of spontaneous remission. A short course of steroid therapy is recommended for the patient with significant symptoms that have not remitted after 7 to 10 days of observation and serial lumbar punctures. Shunting is considered in refractory patients or when loss of vision is threatened.