Objective: Proliferating trichilemmal tumor is uncommon tumors that develop from the hair follicle's outer root sheath. They frequently correspond to the transition of a simple trichilemmal cyst, and they typically occur on the scalp of elderly women. However, multiple proliferating trichilemmal tumor are more rare and difficult to diagnose. Therefore, the diagnosis and treatment of this disease were discussed based on the relevant literature and the clinical data of multiple proliferating trichilemmal tumor in our hospital. Methods: The clinical data of a 74 year old female patient with multiple proliferating trichilemmal tumor and positive family history admitted to department of dermatology were retrospectively analyzed, combined with relevant literature review and discussion. Results: After surgical radical treatment, it is suggested to be multiple proliferating trichilemmal tumor based on the pathological result and immunohistochemical results. Conclusion: Proliferating trichilemmal tumors a rare but morphologically distinctive tumor. The diagnosis is mainly based on pathological results. Based on the histopathologic findings of tumor, this case was diagnosed as proliferating trichilemmal tumor. Surgery is the first choice for treatment. The patient's condition was successfully treated with total excision. Regular postoperative review is required. Proliferating trichilemmal tumor need to be diagnosed and treated using a multidisciplinary strategy. Close clinical follow-up is the most efficient method of early metastasis and recurrence detection.