An exceptional case of nevoid basal cell carcinoma (NBCC) syndrome occurring with acrokeratosis verruciformis (AKV) in the same patient is presented. The occurrence of two rare autosomal dominant diseases in a patient suggests that AKV might be one of the different dyskeratoses of the NBCC syndrome. The clinical aspect of the flesh-colored papules of AKV, indistinguishable from flat warts, might nevertheless prompt performance of a biopsy.