Thyroglossal duct carcinoma (TGDC) is a rare disease with few reported series. No clear consensus exists regarding further management after adequate excision of the cyst, especially the role of total thyroidectomy and postoperative radioiodine therapy. We review our experience of nine cases and the literature to clarify the management of this rare condition. A retrospective study over a period of 10 years was performed using the clinical records from Christian Medical College, Vellore to identify patients with TGDC and to assess the frequency of cases with concomitant papillary carcinoma of the thyroid. The clinical presentation, fine needle aspiration cytology (FNAC), imaging, treatment and follow-up were analyzed. There were a total of nine cases of TGDC with five males. Imaging available in six patients detected a suspicious lesion in four cysts and three thyroid glands. Preoperative FNAC detected atypical cells in two of five cases. Thyroid carcinoma was seen in four (44.4%) after histopathological evaluation. Ultrasound of the neck and image-guided FNAC of the cyst may be adequate initial investigation for thyroglossal cysts. FNAC by itself is not a good investigation to diagnose TGDC as rate of false-negatives and inadequate specimens were high. Solid components and calcification on imaging were predictive of carcinoma within a thyroglossal cyst in 100% cases. All patients with TGDC may be offered total thyroidectomy as a simultaneous or staged procedure to address the high incidence of concomitant thyroid foci of cancer. The role of adjuvant therapy is still debatable.