Trichilemmoma is a benign neoplasm with differentiation towards pilosebaceous follicular epithelium or outer root sheath. It often presents with solitary lesions but when multiple can be associated with Cowden syndrome with various risks of neoplasms. It is commonly found in the region of the head and neck. It is often asymptomatic and long-standing. The treatment is optional but malignancy has to be excluded in the rapidly growing lesion. We are hereby presenting the occurrence of trichilemmoma in a rare location over the genital area. Initial biopsy was suggestive of features of trichilemmoma. She underwent total excision due to fear of malignancy and cosmetic reasons. The histopathology findings corroborated with trichilemmoma. The patient is asymptomatic without any local complaints and recurrence on follow-up for 6 months. Trichilemmoma should be kept as a differential diagnosis in the verrucous lesions over the genital skin.
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