We report a case of acinic cell carcinoma of the parotid gland with neuroendocrine differentiation. Light microscopically, the tumor appeared as clear cell-type acinic cell carcinoma. In addition, the tumor showed neurosecretory features such as Grimelius positivity and the presence of neurosecretory granules by electron microscopy. We suggest that a tumor cell arising from a stem cell can show simultaneous differentiation to both neuroendocrine and acinic cells.