Thyroglossal duct cyst is the most common developmental anomaly of the thyroid and the second most common pathology among childhood cervical masses. They present non-tender palpable mass on midline neck, and they are usually asymptomatic. The cysts are usually benign, but one percent of cases is associated with malignancy. From the world literature, 115 cases of malignant thyroglossal duct cyst are available for review. Despite aspiration cytology and the radiologic images, malignant thyroglossal duct cysts are usually comfirmed after surgery. The authors experienced a case of papillary carcinoma arising from the thryroglossal duct cyst without evidence of local invasion.