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Acinic cell carcinoma (ACC) is a very rare primary tracheal carcinoma. We report a case of primary ACC of the trachea, which represents, to the best of our knowledge, only the fifth such case ever documented. Pharyngolaryngoesophagectomy was necessary for complete resection because the tumor invaded the trachea widely, up to the cricoid cartilage and involving the cervical esophagus. Although invasion of the upper aerodigestive tract by neoplasma is rare, to achieve complete resection and an acceptable prognosis, even a tumor with low malignancy requires wide resection.

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