Neurotrophic receptor tyrosine kinase (NTRK) gene fusions are oncogenic drivers in multiple solid tumors, and they are also important clinical biomarkers. NTRK fusion-positive thyroid cancer (TC) exhibits distinctive clinicopathological features. Patients with advanced, metastatic, or iodine-refractory TC should receive evaluation of NTRK fusion status. However, there have been no authoritative recommendations or standardized procedures for the diagnosis and treatment of NTRK fusions in TC. This review discusses the research advances regarding NTRK fusion-positive TC, summarizes the clinicopathological features of the disease, and outlines the current statuses of NTRK fusion detection and targeted therapeutic agents.