While ectopic thyroid is an uncommon congenital condition, the occurrence of malignant transformation within ectopic lingual thyroid tissue is exceptionally atypical. We report a rare case of not otherwise specified (NOS) thyroid carcinoma (TC) arising in a sublingual ectopic thyroid in a 54-year-old male. The patient presented with progressive dysphonia, dysphagia, and upper airway obstruction, accompanied by severe hypothyroidism. Imaging studies, including ultrasonography, thyroid scintigraphy with technetium-99m pertechnetate, and cross-sectional computed tomography (CT), confirmed the absence of orthotopic thyroid tissue, identifying a highly vascularized sublingual tumor. Preoperative selective arterial embolization was performed to minimize intraoperative bleeding, followed by a successful surgical excision of the described mass. Histopathological analysis revealed NOS carcinoma arising in ectopic lingual thyroid tissue. The patient was subsequently maintained on suppressive Levothyroxine therapy and referred for radioiodine treatment. This case highlights the importance of a multidisciplinary diagnostic and therapeutic approach in the treatment of ectopic lingual TC. To our
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