A 74-year-old man presented with a tumor on his scalp that he had since birth. The authors observed a verrucous pink crusted plaque. The tumor was clinically diagnosed as nevus sebaceus and was removed. Histopathologic study showed, arising from nevus sebaceus, a lobular neoplasm with the classic features of trichilemmoma. Desmoplasia was present in the center of the trichilemmomatous area. This description is typical of a special, uncommon type of trichilemmoma that histologically mimics invasive carcinoma.

Rubinstein-Taybi syndrome is characterized by the presence of a peculiar facies, mental retardation, and broad thumbs and great toes. Several associated cutaneous abnormalities have been reported with this syndrome. Ulerythema ophryogenes is a form of follicular keratosis associated occasionally with other ectodermal defects and congenital anomalies. We describe a 9-year-old child with Rubinstein-Taybi syndrome and ulerythema ophryogenes. This association has not been described previously to our knowledge.