Renal cell carcinoma (RCC) is a heterogeneous group of cancers originating from renal epithelial cells, accounting for over 90% of kidney cancers. Clear cell RCC (ccRCC) is the most common subtype and the focus of this primer. Recent advances in cancer genomics have revealed marked intratumour and inter-tumour heterogeneity, which could have prognostic, predictive, and therapeutic implications. Localized RCC can be managed with surgery, while metastatic RCC is refractory to conventional chemotherapy. However, significant progress has been made in the treatment of metastatic RCC over the past decade, with targeted agents such as sorafenib, sunitinib, pazopanib, axitinib, bevacizumab, and immunotherapies like nivolumab being approved. This primer provides an overview of the molecular biology of RCC, with a focus on ccRCC, and discusses updates to clinical guidelines and potential future directions for research and therapy. Key areas covered include epidemiology, mechanisms/pathophysiology, diagnosis, screening, prevention, management, quality of life, and outlook.Renal cell carcinoma (RCC) is a heterogeneous group of cancers originating from renal epithelial cells, accounting for over 90% of kidney cancers. Clear cell RCC (ccRCC) is the most common subtype and the focus of this primer. Recent advances in cancer genomics have revealed marked intratumour and inter-tumour heterogeneity, which could have prognostic, predictive, and therapeutic implications. Localized RCC can be managed with surgery, while metastatic RCC is refractory to conventional chemotherapy. However, significant progress has been made in the treatment of metastatic RCC over the past decade, with targeted agents such as sorafenib, sunitinib, pazopanib, axitinib, bevacizumab, and immunotherapies like nivolumab being approved. This primer provides an overview of the molecular biology of RCC, with a focus on ccRCC, and discusses updates to clinical guidelines and potential future directions for research and therapy. Key areas covered include epidemiology, mechanisms/pathophysiology, diagnosis, screening, prevention, management, quality of life, and outlook.