Purpose: Nevus sebaceus is a congenital hamartomatous lesion, typically involving head and neck. Various benign and malignant neoplasms can develop in association with nevus sebaceus. We report a case of simultaneous occurrence of squamous cell carcinoma and basal cell carcinoma in nevus sebaceus. Methods: A 73-year-old man presented with erythematous to black verrucous nodules on the right subauricular area. The upper part was accompanied with inflammation and ulceration, and no specific findings suspicious for malignant degeneration were found in the lower part preoperatively. The mass was totally excised and the defect was directly closed. Lymph nodes were not involved on concomitant neck dissection. Results: Histopathologic examination confirmed the presence of squamous cell carcinoma in the upper part and basal cell carcinoma in the lower part of a nevus sebaceus. Negative margins were achieved on resection. No clinical problems were found during the 3-month follow-up period. Conclusion: We experienced a rare case of simultaneous occurrence of squamous cell carcinoma and basal cell carcinoma within the same nevus sebaceus. Because patients with nevus sebaceus have risk of malignant changes, surgical excision and work-up for recurrence and metastasis should be considered in suspicious cases. And even in a totally asymptomatic case, the possibility of occult secondary cancer should be informed before surgery in aged patients.