Thyroglossal duct cyst (TDC) commonly occurs in the neck just below the hyoid bone. Uncommon sites of TDC have been documented, and of these, an intra-thyroid location is very rare. We report such a rare intrathyroid TDC (ITTDC) initially identified by ultrasound examination as an incidental thyroid imaging reporting and data system (TI-RADS) three lesion in the left thyroid lobe of a 59-year-old male patient with primary hyperparathyroidism due to a parathyroid adenoma. The preoperative ultrasound-guided fineneedle aspiration biopsy (US-FNAB) cytology of the thyroid lesion was interpreted as Bethesda III (atypia of undetermined significance or follicular lesion of undetermined significance). A left hemithyroidectomy and left superior parathyroidectomy were performed. The postoperative histology revealed the thyroid lesion to be an ITTDC. An incidental papillary thyroid microcarcinoma was also histologically revealed. The 2.5-year postoperative follow-up was uneventful. Based on literature searches, the clinical features, fine-needle aspiration biopsy (FNAB) cytology, histology, differential diagnosis, treatment, and follow-up of ITTDC were reviewed and discussed. A proposal to categorize ITTDC into two anatomical location subtypes is made. The liability of ITTDC to be misinterpreted on FNAB cytology due to rarity and lack of morphological specificity is emphasized.