Background: Oncocytic carcinoma (OC) arising in the submandibular gland is an unusual malignant neoplasm, with <20 cases previously reported. The cancer is characterized by numerous morphologically abnormal mitochondria present in the cytoplasm and marked cellular pleomorphism. At its most severe, the tumor may invade into the surrounding tissues, including intravascular, lymphatic, or perineural invasion, and lead to regional nodal or distant metastasis. Methods: The current study describes a novel OC case in a 46-year-old male, the youngest case of the review. The patient presented with a 5-month history of an intermittently painful mass. Results: Following magnetic resonance imaging, excisional biopsy, hematoxylin-eosin staining, phosphotungstic acid-hematoxylin staining, and immunohistochemical examination, an OC of the submandibular gland was diagnosed. Conclusion: The current study summarizes the pathogenesis, diagnosis, therapeutics, and the prognosis of OC. The literature review regarding this rare disease is also presented to emphasize the lack of specific markers of OC and the risk of cervical lymph metastasis. Abbreviations: CK = pan-cytokeratin, EMA = epithelial membrane antigen, FNA = fine-needle aspiration, OC = oncocytic carcinoma, PTAH = phosphotungstic acid-hematoxylin, SMA = a-smooth muscle actin.