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Acinic cell carcinoma (ACC) is a low or intermediate-grade malignant epithelial neoplasm of the salivary glands that generally shows an indolent behavior. Most cases arise in the major salivary glands, especially the parotid gland. ACC is usually known as a low-grade malignant tumor; however, poorly differentiated and high-grade transformed variants have been reported and may tend to be late recurrence and metastasis. Our case was a 49-year-old woman with a history of a right parotid rapidly growing mass lesion, which was cytologically diagnosed as monomorphic adenoma on fine-needle aspiration (FNA). Finally, the diagnosis of ACC with dedifferentiated components was reported, followed by parotidectomy. After 30 sessions of radiotherapy, she presented with hemifacial paresis. An imaging examination of the brain showed intracranial hemorrhage, and she underwent a craniotomy. We performed histopathological and immunohistochemical (IHC) examinations and diagnosed metastatic ACC with dedifferentiated components. Few ACC cases with dedifferentiated components and with aggressive behavior have been reported in the literature , and to the best of our knowledge, this article is the first English report in Iranian population.