The nevus sebaceus of Jadassohn (SNJ) is a hamartomatous disorder of the skin and its adnexa pertaining to the group of "organoid nevi,'' most frequently involving the face and scalp. During adulthood, patients with SNJ have a 10% to 20% risk of the development of cutaneous or adnexal neoplasia, so that prophylactic excision before puberty is recommended by most authors, and tissue expansion is considered to be the best method of reconstruction. It has been largely demonstrated in literature that most of the lesions that have been interpreted as basal cell carcinoma (BCC) are actually examples of primitive follicular induction or trichoblastomas, not authentic BCCs. A literature review on histopathologic findings associated with SNJ and a retrospective chart review of two cases occurring in young females are presented. In one case, the lesion was treated by intraoperative expander-assisted reduction and scalp graft (Case 1); in the other one, a primary closure with adjacent tissue was performed (Case 2). No signs of malignant degeneration or residual pathology have been found. For treatment of the biggest lesions, when preoperative tissue expansion cannot be performed, intraoperative one, transfer of a scalp graft has been shown to be a good reconstructive method. For the smallest lesions, a primary closure with adjacent tissue is sufficient.