Background/Aims: Familial aggregation of end-stage kidney disease (ESKD) has been reported in several studies, most of them carried out in USA. It is uncertain to what extent these findings can be generalized to other populations. The objective of this study was to assess whether familial aggregation of ESKD occurs in Brazil and whether it is influenced by race. Methods: Case-control study including 555 ESKD patients and 595 controls without renal disease. Multivariate logistic regression models were used to assess the association between a history of ESKD in a first-degree relative (Fam-ESKD) and the risk of ESKD. Additionally, the association between Fam-ESKD and race among ESKD cases was analyzed. Results: Twenty-seven cases (4.9%) and 5 controls (0.8%) reported Fam-ESKD (p < 0.001). Fam-ESKD was significantly more frequent among cases in a regression model adjusted for all studied covariates (OR = 5.71, 95% CI = 2.13–15.4; p < 0.001). The frequency of Fam-ESKD among dialysis patients belonging to the white, black and ‘mixed’ racial subgroups was 6.0, 4.3 and 4.0%, respectively (p = 0.62). There was no association between race and Fam-ESKD (p = 0.54) in adjusted regression analyses. Conclusion: Fam-ESKD occurs in Brazil but, as opposed to what has been suggested by American studies, it does not appear to be influenced by race.