Objective: To investigate the diagnosis and clinical treatment of maxillofacial connective tissue hyperplastic trichoepithelioma. Methods: The clinical data of two cases of maxillofacial connective tissue hyperplastic trichoepithelioma were summarized and analyzed along with the literature Results : Two cases of maxillofacial connective tissue hyperplastic trichoepithelioma were male, aged 21 and 30 years. The clinical manifestations were painless pale brown and pale white plaques in the maxillofacial region. The lesion was tough and clear, with no ulcers in the middle depression. The course was 10-16 months, with 1-3 months before medical treatment, and the tumor had a significant history of enlargement. After surgery, the skin was cut 3 mm along the outer circumference of the tumor, and local tissue defects were repaired by the adjacent flap. The pathological report showed that the tumor cells were located in the dermis, and were striped, trabecular or nested. The tiny sac contained fibrous connective tissue proliferation. The tumor cells were amorphous without obvious nuclear division. Immunohistochemical analysis reported bcl-2(-), CK7(-), CK19(-), CD34(+), P63(+), CK56(+), and Ki67(±). The pathological diagnosis was connective tissue proliferative hair epithelial tumor. The patient was followed up for 24 months. There was no recurrence of the tumor, no obvious scarring, and no deformity or dysfunction of the maxillofacial region. Conclusion Pathological and immunohistochemical examination is the basis for the differential diagnosis of maxillofacial connective tissue hyperplastic trichoepithelioma, and surgical removal of tumors is an effective treatment.