Trichoepithelioma is a benign epidermal appendage tumor with follicular differentiation. It occurs as a solitary non-familial and a multiple familial type. The multiple familial trichoepithelioma is a relatively common genetic dermatosis characterized by the presence of many small tumors predominantly on the face, which is inherited in an autosomal dominant pattern. The patient was a 18-year-old girl and presented multiple skin colored, pin head sized papules on the face. Skin biopsy revealed tumor islands composed of basophilic cells, showing peripheral palisading and multiple horn cysts. Also, the karyotype of 45, XO with typical clinical symptoms of Turner's syndrome was detected in this patient.
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