We describe a 13-year-old systemic lupus erythematous (SLE) patient who presented with severe headache. The diagnosis of pseudotumor cerebri (PTC) was confirmed by an increased intracranial pressure and normal neuroimaging studies of the brain, including magnetic resonance (MR) venography. She later developed a Coombs positive anemia, lymphopenia, positive tests for antinuclear antibody (ANA) and anti-dsDNA and a migratory polyarthritis confirming the diagnosis of SLE. IgM type anticardiolipin antibodies were positive in low titer. Since she did not have a demonstrable thromboembolic phenomenon in neuroimaging studies, a diagnosis of antiphospholipid antibody syndrome could not be made and anticoagulant treatment was not given. Treatment with pulse i.v. methylprednisolone followed by oral treatment along with azathioprine produced a rapid and dramatic resolution of the clinical symptoms. PTC may also be a neurological manifestation of childhood SLE and should be considered in the differential diagnosis. We suggest that pulse steroids and azathioprine is an effective treatment for this feature.