Renal cell carcinoma (RCC) accounts for 2%–3% of all adult malignancies, with an estimated 209,000 new cases and 102,000 deaths annually. The incidence of RCC has increased over the years, contributing to a rising mortality rate. Risk factors include active and passive smoking, hypertension, and obesity. Approximately 2%–3% of RCC cases are hereditary, with von Hippel-Lindau (VHL) disease being the most common. Diagnosis is often incidental due to widespread imaging use, with over 50% of RCCs detected this way. Symptoms such as flank pain, hematuria, and palpable masses are common, as are metastatic symptoms and paraneoplastic syndromes. Imaging, particularly CT and MRI, is crucial for diagnosis and staging. A biopsy confirms malignancy, and staging is based on the UICC TNM 2009 system. Risk assessment models, such as the SSIGN and UISS scores, are used to predict outcomes. For localized disease, partial nephrectomy is preferred for tumors up to 7 cm, while laparoscopic radical nephrectomy is used for larger tumors. Locally advanced disease may require open radical nephrectomy. Metastatic disease is managed with systemic treatments like tyrosine kinase inhibitors (TKIs), immunotherapy, and targeted therapies. Sunitinib, pazopanib, and temsirolimus are commonly used first-line treatments. Second-line options include everolimus and axitinib. Radiotherapy and bisphosphonates have limited roles but are used for palliation. Follow-up is essential, with imaging and regular monitoring. No standard follow-up protocol is recommended for early RCC, but CT scans are used for advanced cases. Conflicts of interest are noted among authors. The guidelines emphasize personalized treatment based on risk stratification and histological subtypes.Renal cell carcinoma (RCC) accounts for 2%–3% of all adult malignancies, with an estimated 209,000 new cases and 102,000 deaths annually. The incidence of RCC has increased over the years, contributing to a rising mortality rate. Risk factors include active and passive smoking, hypertension, and obesity. Approximately 2%–3% of RCC cases are hereditary, with von Hippel-Lindau (VHL) disease being the most common. Diagnosis is often incidental due to widespread imaging use, with over 50% of RCCs detected this way. Symptoms such as flank pain, hematuria, and palpable masses are common, as are metastatic symptoms and paraneoplastic syndromes. Imaging, particularly CT and MRI, is crucial for diagnosis and staging. A biopsy confirms malignancy, and staging is based on the UICC TNM 2009 system. Risk assessment models, such as the SSIGN and UISS scores, are used to predict outcomes. For localized disease, partial nephrectomy is preferred for tumors up to 7 cm, while laparoscopic radical nephrectomy is used for larger tumors. Locally advanced disease may require open radical nephrectomy. Metastatic disease is managed with systemic treatments like tyrosine kinase inhibitors (TKIs), immunotherapy, and targeted therapies. Sunitinib, pazopanib, and temsirolimus are commonly used first-line treatments. Second-line options include everolimus and axitinib. Radiotherapy and bisphosphonates have limited roles but are used for palliation. Follow-up is essential, with imaging and regular monitoring. No standard follow-up protocol is recommended for early RCC, but CT scans are used for advanced cases. Conflicts of interest are noted among authors. The guidelines emphasize personalized treatment based on risk stratification and histological subtypes.