Aseptic meningitis as an initial and isolated manifestation of systemic lupus erythematosus (SLE) is extremely rare. About a third of patients with SLE have neuropsychiatric manifestations; however, less than 2% develop aseptic meningitis. Therefore, SLE as a cause of aseptic meningitis is commonly overlooked and leads to delayed or even missed diagnosis. We report a case of aseptic meningitis that was later discovered to be SLE and where instituting appropriate treatment led to clinical improvement.