A 34-year-old man with X-linked infantile hypogammaglobulinemia, bronchiectasis, and chronic liver disease had a papular eruption on the trunk and upper extremities. A biopsy specimen revealed caseating granulomas, but special stains, cultures, and electron microscopy failed to reveal an infectious organism. Immunohistochemistry showed that the lymphocytes within the granulomas were almost exclusively of the CD8 + cytotoxic/suppressor T phenotype. Phenotypic analysis of the circulating lymphocytes showed normal numbers of CD4 + (helper/inducer) and CD8 + T cells, whereas B cells were undetectable. Other examples of noninfectious granulomatous disease have been reported in patients with primary hypogammaglobulinemia, but this is the first case of caseating cutaneous granulomatous disease to be reported in a patient with X-linked infantile hypogammaglobulinemia.