ObjectiveTo study the clinical features of and formulate reasonable treatment scheme for thyroglossal duct carcinoma via summarizing the diagnosis and treatment of this disease with literature review.MethodsClinical data including the diagnosis and treatment process, imaging characteristics and individualized therapy of 2 patients with thyroglossal duct carcinoma were analyzed retrospectively.ResultsBoth patients underwent conventional Sistrunk operation based on the preoperative diagnosis of thyroglossal duct cyst. Postoperative pathology confirmed as thyroglossal duct papillary carcinoma in one case, and thyroid papillary carcinoma in another. The latter one was clinically diagnosed as papillary carcinoma of thyroglossal duct with combination of pathological and intraoperative findings. With normal postoperative thyroid function and without clear evidence of malignancy by thyroid ultrasonography, neither received thyroid surgery. 8year followup showed neither local recurrence nor metastasis in one case without any supplementary treatment, and oneyear followup revealed no recurrence in the other one receiving postoperative thyroid hormone suppression therapy.ConclusionsThyroglossal duct carcinoma is often misdiagnosed preoperatively as thyroglossal duct cyst for its clinical rarity. Preoperative ultrasonography and enhancement computerized tomography contribute to diagnosis. The diagnosis depends upon postoperative pathological examination. Operation plan should be formulated comprehensively. Pathological classification decides its prognosis.