Treatment: If treatment is undertaken, complete surgical excision is the treatment of choice. This not only removes the lesion but also removes the risk of malignant potential. Another approach is to watch and wait, with routine observation. If the nevus sebaceus develops any areas of change, a prompt biopsy is warranted. The timing of the surgical removal is controversial, and because the risk of malignancy is low, it is acceptable to wait until the patient is old enough to make the decision. The size and location of the nevus sebaceus dictates the type of surgical excision and repair required. Treatment of the rare nevus sebaceus syndrome requires a multidisciplinary team approach.