A patient with systemic lupus erythematosus who developed pneumococcal epiglottitis is described and the literature reviewed. This infection is extremely rare in adults, and only 10 cases, none of them with SLE, have so far been reported. Epiglottitis is usually caused by Haemophilus influenzae. However, in immunocompromised hosts the probability of Streptococcus pneumoniae as the infecting agent is considerable. Penicillin should therefore be part of the antimicrobial regimen in such patients.