Small cell carcinoma (SmCC) arising in salivary gland is extremely rare, and most of the primary lesions occur in the parotid glands. We report a case of SmCC arising in the palatine gland in a 64-year-old man. The patient had an ulcerated tumor, measuring 35 × 20 mm, in the left side of the palate. A number of enlarged lymph nodes were recognized in the left cervical region. No distant metastases or other tumors were detected on ¹⁸F-fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT). A biopsy was performed from the palatal lesion, and the histopathological diagnosis was SmCC. The patient received radiotherapy alone, because chemotherapy had an increased risk of severe adverse events due to the presence of chronic kidney failure. Radiotherapy was administered to the left palatal and cervical regions in a total dose of 60 Gy. The tumor and the metastatic lymph nodes markedly shrank after the radiotherapy. However, 3 months later, metastatic cervical lymph nodes appeared in the right cervical region. Bony metastases to the right ilium and the left pubis were also detected on FDG-PET/CT. Additional radiotherapy was administered (60 Gy to the right cervical lymph nodes and 40 Gy to the metastatic bone lesions), but was not effective. Regrowth of the primary tumor and bilateral metastatic cervical lymph nodes appeared, and skin, lung, and liver metastases were identified. Finally, the patient died of multiple organ failure 12 months after the initial diagnosis.