Abstract: Carbonic anhydrase (CA) Ⅸ is markedly upregulated in clear cell renal cell carcinoma (ccRCC) as a consequence of abnormal accumulation of hypoxia-inducible factor 1α. CAⅨ plays a crucial role in regulating the intra- and extracellular pH of tumor cells, which is associated with tumor proliferation, invasion, metastasis, and resistance to radiotherapy and chemotherapy. Consequently, CAⅨ represents a potential target for radionuclide imaging in ccRCC. This article reviews the clinical application research and progress of CAⅨ-targeted radionuclide imaging in ccRCC, and discusses its future prospects as an anti-tumor target. The authors conclude that developing CAⅨ-targeted radiopharmaceutical imaging agents holds promise for providing a novel approach to the early diagnosis and effective treatment of ccRCC.