Thyroglossal duct carcinoma is very rare, being present in about 1% of all patients with thyroglossal duct cysts. The clinical presentation of thyroglossal duct carcinoma is very similar to that of its benign counterpart; thus, a diagnosis of malignancy is usually made on postoperative histopathological examination. In general, the Sistrunk operation is sufficient for patients with no signs of invasion. However, total thyroidectomy and appropriate neck dissection are required if there are intra-thyroidal lesions or evidence of cervical lymph node metastasis. We experienced a case of 50-year-old female patient with a papillary carcinoma arising in the thyroglossal duct cyst and subsequent metastases to lateral neck nodes.