BACKGROUND: Kidney cancer is a major urological disease globally, with more than 400 000 new cases diagnosed every year. OBJECTIVE: To investigate incidence and mortality trends for kidney cancer and their associations with modifiable risk factors for kidney cancer. DESIGN, SETTING, AND PARTICIPANTS: The most up-to-date figures on kidney cancer incidence and mortality were collected from the GLOBOCAN database and the Cancer Incidence in Five Continents (CI5). Data on total alcohol consumption and the prevalence of smoking, overweight, diabetes, and hypertension were extracted from the World Health Organization Global Health Observatory data repository. OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS: Age-standardized rates (ASRs) for incidence and mortality and their correlations with potential risk factors for kidney cancer were investigated. Multivariable linear regression analysis was also conducted. The 10-yr temporal patterns for incidence are presented as the average annual percent change with 95% confidence interval using joinpoint regression analysis. RESULTS AND LIMITATIONS: Globally, there is wide variation in kidney cancer incidence and mortality. There were positive correlations between rates of smoking, alcohol consumption, and overweight and ASRs of kidney cancer incidence and mortality. Multivariable regression analysis revealed that alcohol consumption and overweight were significant risk factors for kidney cancer incidence, while smoking and alcohol consumption were significant risk factors for kidney cancer mortality. There was an increasing trend for the incidence of kidney cancer globally, with a particularly prominent trend for European countries. Of note, increasing incidence of kidney cancer is evident even for younger individuals aged <50 yr. However, cancer registries vary by country and period and there is a lack of data regarding the severity of risk factors and disease characteristics such as the distribution of histological groups, tumor grading, and staging. CONCLUSIONS: There is an increasing trend for kidney cancer incidence globally, particularly in European countries and the younger population. Modifiable risk factors for kidney cancer incidence and mortality have been identified. The increasing incidence of kidney cancer among younger individuals is worrying and warrants early action on possible preventive measures. PATIENT SUMMARY: The incidence of kidney cancer has been increasing globally, particularly in European countries and the younger population. Risk factors include smoking, alcohol consumption, overweight, and hypertension, and these factors are all modifiable.