A 19-year-old male had chronic mucocutaneous candidiasis with typical granuloma on the face, scalp and genital area. He had defects in cell-mediated immunity (cutaneous anergy to common antigens, but normal number of T lymphocytes and normal lymphocyte transformation with phytohemagglutinin), and there were no endocrine deficiencies. He had systemic involvement, including lungs, kidneys, liver and spleen diagnosed by radiologic studies. In the histologic findings of the skin, typical features were seen of candida granuloma lesions, and hyphae and spores of Candida albicans also were identified in the Malpighian layer. Therapy with 5-fluorocytosine resulted in a remarkable improvement.