Abstract: Proliferating trichilemmal cyst is also known as proliferating trichilemmal tumor,which is a mass protruding from the surface of the skin caused by the abnormal proliferation of cells in the outer root sheath of the hair follicle due to mutations in the relevant genes in the body. More than 90% of the lesions occurred in the scalp. And it also occurred in the limbs,ears, and other parts. Occurred in the maxillofacial region is rare. A case of proliferating trichilemmal cyst in the maxillofacial region admitted to our department is reported.

Key words: maxillofacial, proliferating trichilemmal cyst, malignant proliferating trichilemmal tumor