Hydatid disease, also known as echinococcal disease, is a zoonotic disease caused by Echinococcus infection. Hydatid cysts of the thyroid are rare. The present case study reports on a patient with hydatid cyst in the thyroid with tracheal fistula. A 54-year-old male patient without any history of exposure to farm animals was hospitalized due to fever and productive cough with occasional hydatid daughter cyst. The patient was diagnosed with a hydatid cyst of the thyroid gland with tracheal fistula by relevant examination. An operation was performed to remove the hydatid cyst and to repair the fistula. The patient took oral albendazole after surgery to prevent hydatid recurrence. Operative recovery was uneventful and the patient resumed his normal activities. Prompt diagnosis and an appropriate surgical treatment prevented a potentially fatal outcome. Furthermore, the characteristics of thyroid cystic echinococcosis, and its diagnosis and treatment in the present case and other cases reported in the literature were summarized and reviewed. Although the thyroid gland is rarely affected, hydatid cyst disease should be considered as a differential diagnosis of cystic lesions of the thyroid gland in patients living in regions where hydatid cyst disease is endemic.