Kidney cancer globally accounts for more than 131,000 deaths each year and has been found to place a large economic burden on society. However, there are no recent articles on the burden of kidney cancer across the world. The aim of this study was to present a status report on the incidence, mortality and disability-adjusted life years (DALYs) associated with kidney cancer in 195 countries, from 1990 to 2017. Vital registration and cancer registry data (total of 23,660 site-years) were used to generate the estimates. Mortality was estimated first and the incidence and DALYs were calculated based on the estimated mortality values. All estimates were presented as counts and age-standardised rates per 100,000 population. The estimated rates were calculated by age, sex and according to the Socio-Demographic Index (SDI). In 2017, kidney cancer accounted for 393.0 thousand (95% UI: 371.0–404.6) incident cases, 138.5 thousand (95% UI: 128.7–142.5) deaths and 3.3 million (95% UI: 3.1–3.4) DALYs globally. The global age-standardised rates for the incidence, deaths and DALY were 4.9 (95% UI: 4.7–5.1), 1.7 (95% UI: 1.6–1.8) and 41.1 (95% UI: 38.7–42.5), respectively. Uruguay [15.8 (95% UI: 13.6–19.0)] and Bangladesh [1.5 (95% UI: 1.0–1.8)] had highest and lowest age-standardised incidence rates, respectively. The age-standardised death rates varied substantially from 0.47 (95% UI: 0.34–0.58) in Bangladesh to 5.6 (95% UI: 4.6–6.1) in the Czech Republic. Incidence and mortality rates were higher among males, than females, across all age groups, with the highest rates for both sexes being observed in the 95+ age group. Generally, positive associations were found between each country’s age-standardised DALY rate and their corresponding SDI. The considerable burden of kidney cancer was attributable to high body mass index (18.5%) and smoking (16.6%) in both sexes. There are large inter-country differences in the burden of kidney cancer and it is generally higher in countries with a high SDI. The findings from this study provide much needed information for those in each country that are making health-related decisions about priority areas, resource allocation, and the effectiveness of prevention programmes. The results of our study also highlight the need for renewed efforts to reduce exposure to the kidney cancer risk factors and to improve the prevention and the early detection of this disease.