Chronic mucocutaneous candidiasis is a chronic infection of mucoses, nails and skin due to Candida sp., that does not respond to standard treatment. We report on three patients with chronic mucocutaneous candidiasis in association with iron deficiency, IgA deficiency in one patient and adult onset in another. We review the clinical manifestations, the classification and the treatment of this disorder. The immunologic defects associated with chronic mucocutaneous candidiasis provide a model to study the mechanisms of defense of the host against fungal infections.