Cryptococcal meningitis is a rare but well recognized complication of systemic lupus erythematosus (SLE). Since in all previously reported cases in the medical literature the patients developed this opportunistic infection as the result of immunosuppressive therapies, whether the intrinsic immunological abnormalities of SLE per se contribute to the susceptibility remains controversial. We report on a patient who presented concurrently with cryptococcal meningitis and cryptococcaemia at the time of her diagnosis of active SLE. This highlights the possibility that intrinsic immunological defects of SLE may be directly responsible for the predisposition to fungal infections. In addition, when SLE patients present with neurological symptoms, the possible presence of central nervous system (CNS) infection must be checked for, even if immunosuppressive treatment is not being considered.