Large cell undifferentiated carcinoma is a rare malignant neoplasms of the parotid gland. Final diagnosis is according to the histopathological features of this tumor, and a primary location of the lung should be ruled out. Surgery is the main treatment modality and postoperative radiotherapy is needed. The prognosis seems to be poor. A 66-year-old woman presented with a right infra-auricular mass in January, 2008. Head and neck computed tomography revealed a high-dense parotid mass with several satellite lymph nodes. Radical parotidectomy was performed and immunohistochemistry revealed large cell undifferentiated carcinoma. Postoperative radiotherapy was performed and no recurrence of tumor or neck metastasis was found. We review herein the clinicopathologic features, histopathology, treatment and prognosis.