Squamous cell carcinoma (SCC) is the most common head and neck malignancy. However, squamous cell carcinoma rarely presents in major salivary glands, especially in the submandibular gland. In the literatures, most cases of SCC of the submandibular gland are metastasis from another body sites, including skin, lymph nodes and organs of upper aerodigestive tract. The incidence of primary SCC of the submandibular gland is extremely rare. So, the therapeutic strategy of this malignancy is still unclear. Recently, we experienced a case of primary SCC in the left submandibular gland who having long-standing submandibular gland sialolithiasis. The patient was treated with surgery and postoperative radiotherapy.