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Background: Primary sebaceous carcinoma of the parotid gland is extremely rare, and because of its rarity, clinicopathological characteristics and histogenesis are not fully understood. Methods: Here, we report a patient who presented with a left infra-auricular painless mass. We present the histological features and discuss possible optimal treatments based on previous literature. Results: The mass was suspected to be a myoepithelial tumor or possibly a pleomorphic adenoma. Initially, the mass was resected with preservation of the facial nerve, but this caused facial palsy. Because the histological examination showed a sebaceous carcinoma and a part of the mass could be remaining on the facial nerve, additional surgery was performed, and the facial nerve was reconstructed with cervical nerve. Follow-up after 7 months showed no sign of recurrence of metastasis. Conclusion: We encountered a rare sebaceous carcinoma of the parotid gland. Additional surgery was performed because preoperative diagnosis was difficult.