Thyroglossal duct cyst (TGDC) is the most common congenital thyroid developmental anomaly and also the most common congenital neck anomaly. Incidence of TGDC in the adult population is estimated at 7 %. However, malignant changes are rare and occur in less than 1% of TGDC. Over 90% comprises papillary types while squamous cell carcinoma comprises 4.3%. Here we report the case of a 52-year-old female with unexpected finding of papillary carcinoma arising from TGDC. Subsequent total thyroidectomy and radioactive iodine treatment is performed.