A case of adenoid cystic carcinoma located in trachea, primarily wrongly recognized as a thyroid cancer is presented with an update of the literature. The patient underwent surgical treatment (total thyroidectomy, and partial resection of trachea). Histopathological examination found trachea as a primary location of ACC and thyroid tumour as a metastatic one. After operation the patient underwent radiotherapy. Literature review found ACC of trachea as a very rare malignant tumour, and no such a case was presented in the past.