Clear cell renal cell carcinoma (ccRCC) represents the predominant histological subtype of renal cell carcinoma (RCC), constituting approximately 85% of all RCC cases. Recent advancements in therapies aimed at targeting angiogenesis have marked a significant breakthrough in the treatment of ccRCC, with several of these therapies receiving approval for clinical use. Furthermore, the introduction of immune checkpoint inhibitors (ICIs) has demonstrated efficacy in the management of ccRCC. Nonetheless, there is an urgent need for the identification of predictive and prognostic biomarkers, which are currently under investigation. This review offers an extensive examination of the pathological, genomic, and molecular characteristics of ccRCC, with particular emphasis on its immune attributes. Additionally, it addresses the clinical implications of targeted therapies and immunotherapies, whether administered as monotherapy or in combination with traditional or novel agents, while also evaluating the results of pertinent clinical trials. By encompassing a wide range of topics related to ccRCC, from foundational knowledge to clinical applications, this review aims to deepen the understanding of the essential features of ccRCC and to establish a theoretical basis for the formulation of clinical strategies.