Objective: To assess whether increased intracranial pressure (IIP) is associated or coincidental with systemic lupus erythematosus (SLE) and to characterize the patients with this condition, its commonly used treatment, and outcome. Methods: A retrospective cohort study was conducted at the Hadassah Medical Center, Jerusalem, Israel. Files were retrieved from the hospital archives by screening records from 1980 to 2006 using the terms "SLE" and "pseudotumor cerebri" or "benign intracranial pressure." Medical records were screened for data regarding: (1) The prevalence of IIP among hospitalized patients; (2) the demographic, clinical, and laboratory characteristics of SLE patients reported to have at least 1 episode of IIP. Results: Ten of 651 hospitalized SLE patients (prevalence, 1.5%) were found to have IIP, exceeding the prevalence of 1 to 19 per 100,000 reported in the general population. In 3 patients this was a presenting manifestation of SLE and the average time elapsed until IIP diagnosis was 10.7 months. Arabs were strikingly overrepresented among the 10 individuals, comprising 6 patients (versus 19% Arabs of all SLE patients). In 8 patients, other central nervous system (CNS) abnormalities were found either clinically or in laboratory tests. Two individuals had elevated antiphospholipid antibodies (aPL) and a history of thromboembolic events, compatible with antiphopholipid syndrome. The most commonly employed treatments were corticosteroids and acetazolamide, each in 9 patients with generally good clinical response. Conclusions: We propose that IIP is a manifestation of SLE and that corticosteroids should be considered as first-line treatment.