The thyroid descends through the foramen cecum leaving the thyroglossal duct, which disappears between the fifth and the tenth week of pregnancy. The lack of involution of any part of this duct results in thyroglossal cyst formation. Its diagnostic approach is made by cervical ultrasound, computed tomography and magnetic resonance imaging. Approximately 1 % of the thyroglossal cyst formation contains malignant elements, and the most reported primary tumor has been papillary carcinoma. The recommended treatment for these carcinomas is controversial and it has evolved as time goes by. From Sistrunk procedure to neck dissection with total thyroidectomy and complementary therapies, such as iodine ablation and thyroid supplements, yet there is still no consensus as to the type of surgery and postoperative management it should be used to treat this carcinoma. Therapy should be applied according to each specific case, and it should be based on histological diagnosis, the invasive character of the tumor, and the lymph node affectation. In this paper we review the literature published so far with regards to the treatment of this carcinoma.