A clinical and histologic review of 22 patients (13 males, 9 females) with cutaneous lesions classified as desmoplastic trichilemmoma is reported. Typically, the lesions occur as solitary dome-shaped papules on the face during (at least) the fifth decade of life. The average age was 64 years (range 19-89) with a median age of 66.5 years. The most frequent clinical diagnosis was basal cell carcinoma, and the most common sites were the lip, eyebrow and nose. Two lesions occurred in examples of nevus sebaceus of Jaddasohn. The proportion of desmoplasia varies, but is generally between 20% to 60% of the lesion. Ulceration is seen in a minority of lesions but when present, is closely associated with underlying desmoplasia. Desmoplasia may be seen in small, as well as large, trichilemmomas; it generally occurs centrally but at times may be seen peripherally. The appearance of strands of epithelial cells entrapped in dense fibrosis and hyalinization may mimic desmoplastic variants of squamous cell carcinoma and basal cell carcinoma. This appearance may be particularly troublesome when the desmoplasia occurs at the base of the biopsy specimen. Knowledge of this phenomenon and the search for more typical features of trichilemmoma such as clear cells and peripherally palisaded columnar cells upon a thickened, eosinophilic basement membrane will allow the correct diagnosis to be made. We believe that the desmoplasia occurs as a secondary change in pre-existing trichilemmomas.