Proliferating Trichilemmal Tumor (PTT) is a rare tumor of the outer root sheath of the hair follicle with different biological behavior. They usually develop in older patients, most commonly affecting women, from pre-existing pilar cyst. Histologically, this tumor may mimic squamous cell carcinoma (SCC). The standard of treatment is surgical excision with a margin of normal tissue. As most of the patients are not good surgical candidates, Radiotherapy plays an important role in the management of this tumor. We report a case of PTT on the occipital region of a 79-year-old female with its brief overview. In view of large size of the lesion and advanced age of the patient, Neoadjuvant Radiotherapy was planned and delivered. A near complete response of the lesion to radiotherapy was observed with good cosmesis. We discuss the role of radiotherapy in the management of PTT in various settings with literature review. Keywords: Proliferating Trichilemmal Tumor, Proliferating Pilar Tumor, Radical Radiotherapy, Neoadjuvant radiotherapy, outer root sheath tumor, skin cancer, scalp tumor, cosmesis.
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