Pneumocystis carinii pneumonia (PCP) is the most common illness associated with the acquired immunodeficiency syndrome (AIDS) in the United States and also occurs in immunocompromised persons not infected with the human immunodeficiency virus.. Several advances have taken place in the treatment and prophylaxis of PCP, with most clinical trials conducted in patients with AIDS. Treatment of choice is trimethoprim-sulfamethoxazole (TMP-SMX). Desensitization regimens are available for those who have a fever or rash associated with the agent. Patients with severe PCP who cannot tolerate TMP-SMX may be treated successfully with pentamidine or trimetrexate. Those with mild to moderate disease may receive dapsone-trimethoprim, clindamycin-primaquine, or atovaquone if they cannot take TMP-SMX. Adjunctive therapy with corticosteroids improves the outcome in patients with AIDS and severe PCP.