Thyroglossal duct carcinoma is a rare malignant tumor which is usually diagnosed postoperatively. Clinical and pathological features of 3 cases of thyroglossal duct carcinoma were investigated and the optimal treatment protocol was proposed. The clinical presentation of thyroglossal duct carcinoma is very similar to that of its benign counterpart. Two cases were diagnosed as thyroglossal duct cyst prior to the operation, the remaining one as dermoid cyst. All three cases were diagnosed as papillary carcinoma of thyroid origin after microscopic examination. The characteristic histological hallmarks including: formation of papillary structure; nuclear morphological variations such as ground glass nuclei, pseudo-inclusions, intranuclear grooves and filaments; concentrically calcified structures termed psammoma bodies which is regarded as a strong indication of papillary carcinoma;positivity in immunohistological staining for thyroglobin. Sistrunk procedure of excision is the choice for treatment. A close follow-up is needed. In the presence of thyroid gland masses or cervical lymphadenopathy, thyroidectomy or neck dissection should be recommended.