Abstract:Objective To summarize the clinical manifestations, diagnosis, and treatment of pilomatrixoma in the ear and nose.Methods A retrospective analysis was performed on the clinical manifestations, pathological features, treatment and prognosis of 29 cases of pilomatrixoma diagnosed and treated in Shenzhen Children’s Hospital from May 2015 to May 2020.Results Among the 29 cases, only 4 cases were diagnosed as pilomatrixoma preoperatively, and 1 case was diagnosed as hemangioma. The other cases were admitted to the hospital as unknown neoplasms before operation. All tumors or neoplasms of the patients were removed by intubation under general anesthesia. After operation, the neoplasms were diagnosed as pilomatrixoma by histopathological examination. During the follow-up period of postoperation, no recurrence, infection, malignancy and other conditions were observed in all patients.Conclusions Pilomatrixoma in the ear and nose is common, but it is still easy to be confused and misdiagnosed with sebaceous cyst, epidermoid cyst, and hemangioma. Surgical resection is the preferred treatment of this disease with a good prognosis.