Balkan endemic nephropathy (BEN) has remained a geographically constant endemic for 50 years. Despite extensive research, its etiology remains unknown. In the current issue, in a study in one of the earliest sites where the endemic was first recognized, Dimitrov et al. confirm the persistance of the endemic into a new generation and also identify a maternal link in the pathogenesis of BEN. This intriguing finding needs to be confirmed in other endemic areas.