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.