Pneumocystis pneumonia (PCP) is an infection of the lungs caused by the opportunistic fungal genus Pneumocystis. In humans, PCP is a serious and potentially life-threatening infection occurring in immunocompromised individuals, particularly those who have AIDS, or following immune suppression from malignancy, organ transplantation, or therapies for inflammatory diseases. Several recent studies have contributed to understanding of the biology and pathogenesis of the organism yielding new diagnostic approaches and therapeutic targets. Although trimethoprim-sulfamethoxazole remains the mainstay of prophylaxis and treatment, ongoing concerns for emerging Pneumocystis resistance supports the continuing investigation for novel therapeutic agents.