Thyroglossal duct cyst carcinoma (TGDCa) is a rare cause of neck masses in children. Its pathophysiology stems from two main theories. One suggests that the primary carcinoma originated from ectopic thyroid tissue, whilst the other suggest it is a result of metastasis to the thyroglossal duct cyst from the thyroid gland. A case of a 12-year-old girl who presented with a suspicious neck mass and subsequently diagnosed with TGDCa is reported. A literature review was performed and we conclude that aggressive surgical management of a Sistrunk procedure, total thyroidectomy, bilateral tracheoesophageal groove clearance and neck dissection was justified for this child. We found that if the preoperative diagnosis is TGDCa, a more aggressive surgical approach tends to be taken. However, cases of recurrence remain low and a total thyroidectomy does entail a larger morbidity for the pediatric patient. Hence, we do not recommend an aggressive approach routinely. In this review, we extrapolated AJCC data for thyroid malignancies and staged the TGDCa reported in literature in the last 10 years. It showed us that pT3 tumors tend to have a more aggressive surgical course, whilst a pT1 TGDCa tend to receive a more conservative surgical approach.