Background: Nevus sebaceous of Jadassohn is a congenital hamartomatous lesion, which involves sebaceous gland, apocrine, and trichilemmal proliferative changes. It presents three clinical stages; the last one is characterized by epithelial tumors arising. Objective: To determine the frequency and associated tumors type, as well as the clinic and epidemiologic characteristics related with them. Material and method: A retrospective analysis at the Instituto Dermatológico de Jalisco Dr. José Barba Rubio during a five-year period was made. All histopathology reports of nevus sebaceous of Jadassohn were included and three different pathologists in a blind fashion reviewed the slides of tumors arising in nevus sebaceous. Results: We included 260 cases with diagnostic of nevus sebaceous of Jadassohn, mean age 19.7 years, female sex predominance (55%), the most frequent localization was scalp (61.5%). Eleven per cent of the 260 cases were in tumor stage, with predominance in 31 to 40 years old (27.5%). Benign tumors were the most frequent (97%), trichoblastoma predominated (50%); only one case of basal cell carcinoma was found. More than one tumor was associated to nevus sebaceous of Jadassohn in 13.7% of cases. Conclusions: Arising malignant tumors in nevus sebaceous of Jadassohn are uncommon, moreover in pediatric age. Prospective studies are necessary in order to determine the real association with nevus sebaceous of Jadassohn tumors. [ABSTRACT FROM AUTHOR]