Pseudotumor cerebri is a clinical syndrome characterized by elevated intracranial pressure with normal biochemical and cellular characteristics of the cerebrospinal fluid and normal radiologic studies of the skull. This syndrome has been reported infrequently as a primary feature of central nervous system involvement by systemic lupus erythematosus (SLE). We report clinical features in 3 adolescent black females with pseudotumor cerebri as a presenting manifestation of SLE.