Thyroglossal duct cyst is the most commonly encountered midline and upper cervical mass that can also be a rare form of malignancy of about <1%. This is a rare case without well-defined management and staging criteria and as such, it has been a cause of debates regarding optimal management as well as the extent of completeness of surgery from Sistrunk procedure only to Sistrunk procedure with total thyroidectomy.
Conclusion: This is a case report of a rare occurrence of a locally advanced papillary thyroid carcinoma of thyroglossal duct cyst presenting as a benign cervical mass. The management dilemma and ultimate surgical approach was carefully drawn with the patient.