RESUMEN
BACKGROUND: Neuroendocrine tumors mainly occur in the stomach, intestine, pancreas, and lung and are rarely detected in the thyroid. Thyroid neuroendocrine tumors, designated medullary thyroid carcinoma, generally present with elevated calcitonin. Calcitonin-negative neuroendocrine tumors of the thyroid are extremely rare. CASE SUMMARY: Here, we present a case report of a 56-year-old female patient with a neck pain complaint. Total thyroidectomy was conducted after comprehensive evaluation, and diagnosis was confirmed as calcitonin-negative neuroendocrine tumor of the thyroid. Two months later, liver metastasis was detected, and transcatheter arterial chemoembolization was subsequently performed to control growth. However, the curative effect was unsatisfactory and multiple intrahepatic metastases occurred after 3 mo. CONCLUSION: Owing to the rarity of this disease, no clear guidelines are available for treatment. In addition to reporting this rare case, we have reviewed and summarized associated medical literature with an aim to provide a comprehensive reference platform for subsequent research.