The hyper-immunoglobulin E syndrome is a primary immunodeficiency characterized by recurrent pyogenic skin and lung abscesses, dermatitis, and elevated serum immunoglobulin E levels. Pneumocystis jiroveci (formerly Pneumocystis carinii) is not typically associated with hyper-immunoglobulin E syndrome. We identified 7 patients with hyper-immunoglobulin E syndrome with P. jiroveci detected in respiratory or pulmonary pathology specimens. In 5 patients it was the sole pathogen, and in 2 other patients it contributed to a polymicrobial etiology. No consistent prophylaxis was given, and there have been no recurrences on long-term follow-up. Our experience suggests that P. jiroveci can cause pneumonia in patients with hyper-immunoglobulin E syndrome both with and without chronic lung disease.