Conventional imaging is suboptimal at evaluating disease status in renal cell carcinoma (RCC) because of poor sensitivity. Furthermore, there is an unmet need for the treatment of metastatic RCC, both in terms of improvement of progression-free survival and limitation of toxicity. For this reason, radionuclide imaging and radionuclide therapy are extensively investigated. This review provides an overview of the current progress in molecular imaging and radionuclide therapy in clear cell RCC and will focus on promising detection and therapy strategies targeting the carbonic anhydrase IX antigen, which is expressed in clear cell RCC.