Malignant tumors arising in thyroglossal duct remnants are relatively uncommon, occurring in less than 1% of thyroglossal duct abnormalities. Papillary adenocarcinoma constitute 75~85% of all these lesions. Papillary adenocarcinoma is more common in females, and distant metastasis is rare. Wide local excision is the treatment of choice when the neoplasm is confined to the resected cyst contents, and long-term prognosis is very favorable. Recently the authors had experienced one case of papillary adenocarcinoma originating in the thyroglossal duct remnants, which was removed by local excision(modified Sistrunk prodedure), and the result seems to be satisfactory up to date. Related articles were reviewed, and discussions were made.