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Ectopic thyroid gland is usually located along the development path of thyroid in the anterior midline of the neck. However, its occurrence within the mediastinum is very rare. Mediastinal ectopic thyroid cyst is usually benign and asymptomatic, but we experienced a case of the mediastinal ectopic thyroid cyst inducing a severe airway obstruction in a 45-year-old male. The mass had no connection to the cervical thyroid gland and histological examination revealed a mediastinal cyst of an ectopic thyroid. We present the case with a review of the related literatures.