Autosomal dominant transmission of nevus sebaceous of Jadassohn

Background: Nevus sebaceus of Jadassohn is a complex congenital hamartoma that has a predominant sebaceous component. It presents as waxy, well-circumscribed, yellow plaques localized to the head and neck. Its genetic etiology has more often been described as sporadic, with scattered cases featuring autosomal dominant transmission also reported. The development of benign tumors within the lesions is a well-known phenomenon and requires clinical surveillance for new growths suggesting malignant transformation. MAIN Observations: We report three cases of nevus sebaceus of Jadassohn localized to the scalp in a mother and her two daughters. Conclusions: This case further supports the underlying genetic association in familial cases of NSJ, specifically autosomal dominant transmission.