A 4-year-old girl with pachyonychia congenita was followed from birth. During childhood, slow progression of the nail hypertrophy was seen because she was not willing to have the nails ground or cut. After grinding of her nails under general anesthesia, the child has had acceptable nail thickness, and continued regular grinding without anesthesia. The child had recurrent oral and cutaneous candidiasis. Investigations showed a weakly positive response to Candida albicans in the lymphocyte transformation test, and absence of a delayed hypersensitivity skin test response to Candida. The secondary infection due to Candida seemed to prevail because of an immune defect.