Chronic mucocutaneous candidiasis is a disorder of the skin, nails or mucous membranes in the absence of another cause of the infection. It is also associated with autoimmune endocrinopathies in 40% of patients. It is thought to be due to a T-cell defect, although no precise mechanism has been elucidated. There have been two previous cases of Pneumocystis carinii pneumonia reported with this condition. We report a fatal case in a 34-year-old male.