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Trichoepitheliomas are benign hair follicle tumors that typically present as slow-growing sharply circumscribed, symmetrical, domeshaped lesions. They can occur as a solitary lesion or as part of the autosomal dominant multiple familial form. Trichoepitheliomas are predominantly confined to the face with a symmetrical distribution along the nose, cheeks, eyelids, forehead, and preauricular area. Wepresent the first pediatric case of a solitary trichoepithelioma in the external ear canal of 12-month-old girl presenting to the ENT officewith otalgia and an EAC mass noticed by the patient's Mom 10 daysprior. The patient underwent complete excision of a 1.5 cm cyst-likestructure without rupture and an acellular graft was used to cover the defect. Histologic evaluation demonstrated numerous hair follicles, each surrounded by a well-defined perifollicular sheath consistent with a trichoepithelioma. Both clinically and histologically, trichoep -itheliomas are similar to basal cell carcinoma and have a low risk of malignant transformation. Excisional biopsy with complete resection of overlying skin is recommended and should be performed promptly to avoid delay in diagnosis.
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