Familial autosomal dominant cylindromatosis (FADC) is a rare disease characterized by multiple cutaneous cylindromas, trichoepitheliomas, and spiradenomas. We present a case of a woman with scalp lesions and a parotid mass. Biopsy of a skin nodule demonstrated cylindroma, and fine-needle aspiration of the parotid mass suggested membranous basal cell adenoma, which was confirmed following superficial parotidectomy. The study includes case report and review of the literature. Eighteen cases of cylindromatosis with coexistent salivary gland membranous basal cell adenoma were reported. Seventeen cases involved the parotid gland; there are 2 reports of malignant transformation. FADC should be considered in anyone with coexistent dermal and salivary gland neoplasms. Membranous basal cell adenoma may be mistaken for adenoid cystic carcinoma on cytologic or histologic examination. Total parotidectomy with facial nerve preservation or superficial parotidectomy with close follow-up is recommended, as multifocality and malignant transformation exist.