Primary neuroendocrine small cell carcinoma of the parotid gland is a rare malignant tumor, the clinical diagnosis is difficult. This article report a primary parotid neuroendocrine small cell carcinoma which was initially diagnosed of adenoid cystic carcinoma of the parotid gland and was operated, but recurred 2 years later. The artide discuss about the characteristic of this disease and review the relevant literatures.