Chronic mucocutaneous candidiasis (CMC) is usually characterized by onset in childhood and is almost never complicated by deep fungal infection. The authors report two cases of fatal Candida meningitis in patients who suffered from mild, adult-onset CMC. The pedigrees suggest an autosomal recessive disorder. In the index cases and in a symptomatic sibling, the immunologic work-up showed a specific cellular deficit as opposed toCandida albicans, as is typical of other forms of CMC. Both families were of French Canadian descent and originated from eastern Quebec. Three other cases of primary Candida meningitis in patients of the same ethnic origin are also reported and reviewed. It is suggested that these cases may represent a variant of familial adult-onset CMC, in which there is a striking predisposition to deep infection.