Nevus sebaceous of Jadassohn (NSJ) is an inborn, cutaneous hamartoma that is presented as a round-oval, or linear, yellowish-orange hairless plaque with an excess of sebaceous glands, typically localized to the head or neck. NSJ disease progresses slowly in three general stages. Due to its embryological origin, it yields an already documented potential for a variety of epidermal and adnexal tumors. The incidence of secondary neoplasms within NSJ is 10-30%, and the risk of neoplastic transformation increases with age. The majority of neoplasms are benign. Regarding malignant tumors, NSJ is usually associated with basal cell carcinoma. All neoplasms are typically encountered in long-standing lesions. Owing to NSJ’s ample variety of associations with neoplasms, its management demands a case-driven tailored treatment. We present the case of a 34-year-old female with NSJ.