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Parathyroid carcinoma accounts for about 0.5%-5% of all parathyroid neoplasms. Very rarely,but if the intrathyroidal parathyroid gland is present, the carcinoma can arise in that developmentalanomaly. It is very difficult to distinguish a thyroid nodule from an intrathyroidal parathyroidneoplasm with preoperative radiologic and cytologic evaluations. A 59-year-old malewas initially evaluated as presenting a follicular thyroid neoplasm accompanied by hyperparathyroidism. He received hemi-thyroidectomy with central neck dissection and subtotal parathyroidectomy. The final pathology evaluation revealed intrathyroidal parathyroid carcinomaand hyperplasia of contralateral parathyroid glands. We report this very rare and unique clinicalsituation with a literature review.