Basal cell nevus syndrome is a genetically determined disease characterized by 5 major manifestations (multiple basal cell carcinomas, jaw cysts, skeletal abnormalities, pits of the hands and feet, and ectopic calcification) and a variety of developmental anomalies. We present a case of basal cell nevus syndrome in which the patient bad adenoid cystic carcinoma of the minor salivary glands develop in the soft palate resulting in distant pulmonary metastases. The patient died at the age of 44 years of respiratory complications. To our knowledge, this is the first simultaneous occurrence of basal cell nevus syndrome with adenoid cystic carcinoma of salivary gland.
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