Balkan endemic nephropathy (BEN) has attracted increasing attention as a po
ssible environmental disease, and a significant amount of research from com
plementary scientific fields has been dedicated to its etiology. There are
two actual competing theories attempting to explain the cause of this kidne
y disease: 1) the mycotoxin hypothesis, which considers that BEN is produce
d by ochratoxin A ingested intermittently in small amounts by the individua
ls in the endemic regions, and 2) the Pliocene lignite hypothesis, which pr
oposes that the disease is caused by long-term exposure to polycyclic aroma
tic hydrocarbons and other toxic organic compounds leaching into the well d
rinking water from low rank coals underlying or proximal to the endemic set
tlements. We outline the current developments and future prospects in the s
tudy of BEN and differentiate possible factors and cofactors in disease eti
ology.