OBJECTIVE The aim of this study was to examine the ocular fundus pathology in patients with Balkan endemic nephropathy (BN) and chronic kidney diseases (CKD). METHODS The study included 51 patients with BN from the South Morava River region in Serbia, and 102 subjects with different stages of chronic renal diseases, matched according to age and gender, obtained from a database used in a recently published study. All patients had visited Outpatient Department of the Clinic of Nephrology, Clinical Center Nis. All patients underwent routine ophthalmic examinations. RESULTS There were significantly more (P < 0.001) patients with age-related macular degeneration (AMD) in the group with BN (31.37 %) than in those with CKD (5.88 %). Multivariate logistic regression analysis confirmed that the significant factors related to AMD in the group with BN were albuminuria (P < 0.05) and proteinuria (P < 0.05); in CKD patients, the level of HDL (P < 0.05), while negative correlation with the level of triglyceride was registered (P < 0.05). There was no association between estimated glomerular filtration rate and AMD. The significant factors related to retinopathy in the group with BN are age (P < 0.05) and serum creatinine values (P < 0.05), in patients with CKD increasing age (P < 0.001) and DM (P < 0.05). CONCLUSIONS Ocular fundus pathology in patients with BN is similar to the pathology of other CKD, but with significantly more AMD (about four times), probably related to the genetic/epigenetic factors.