Nevus sebaceous of Jadassohn is a hamartoma with a combination of abnormalities of the epidermis, hair follicles, and sebaceous and apocrine glands. Herein, we discuss the results of an extensive literature review on the topic of nevus sebaceous with a particular focus on the debate about the necessity for prophylactic excision. We also focus on the documentation of associated malignant tumors that were reported to develop within NS. In addition to reporting the number and types of neoplasms, we documented the recommendations of all authors for therapeutic handling of these nevi.

We report the unusual case of a young child who developed an idiopathic basal cell carcinoma (BCC). The patient was otherwise healthy, with no history of excessive sun or radiation exposure or any predisposing factors for nonmelanoma skin cancer. This is a rare case that exemplifies the need for pediatricians and dermatologists to be aware that BCC may occur in children, even if they have no predisposing factors.

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