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Introduction: Nevus Sebaceus of Jadassohn (NSJ) is a conjenital hamartomatous lesion which usually involves the face or the scalp. Number of benign and malignant neoplasms can increase. Basal Cell Carcinoma (BCC) is a typical kind of such malignancies. Materials & Methods: Hereby, we report a case of BCC of the scalp arising from NSJ. The lesion was surgically taken away completely. Findings: Histopathologic results has confirmied BCC arising from NSJ. Discussion & Conclusion: Any unnatural growth at the region of head and neck must be considered suspicious and a biopsy is suggested to clarify the case.