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Introduction: IgA nephropathy is the most common form of primary glomerulonephritis in the world and significantly contributes to the development of chronic kidney disease and end-stage renal failure. Objective: To present new insights into IgA nephropathy. Aim: To present new insights into IgA nephropathy. Methods: A review of the published literature is made. Results: The variability of the incidence of the disease among countries in the world is different, it is partly related to non-standardized indications for kidney biopsies in various centers. The frequency of IgA nephropathy is influenced by genetic and environmental factors. The spectrum of clinical manifestations of the disease is wide, the most common being hematuria. The pathogenesis of IgA nephropathy is still not completely clear. The diagnosis was based exclusively on the pathohistological immunofluorescence finding of dominant galactose-deficient IgA1 deposits in the renal mesangium. The Oxford Classification scores five pathohistological features of IgA nephropathy as independent risk predictors of renal disease progression. Therapeutic action is possible for active lesions. The disease is treated with non-immunosuppressive and immunosuppressive drugs. The therapeutic goal in children is to reduce proteinuria below 0.2 g/day/1.73 m2 (or <0.2 g/g creatinine) and to maintain blood pressure below the 50th percentile for age, sex and body height. 15–20 years after the onset of the disease, 20–30% of children develop progressive disease. Elderly age, hypertension, proteinuria, and impaired renal function at the time of diagnosis are poor prognostic indicators. 20–40% of patients develop end-stage renal failure within 20 years of diagnosis. The recurrence of IgA nephropathy in the transplanted kidney occurs in 40–60% of transplanted patients. Conclusion: Although the understanding of the pathogenesis of IgA nephropathy has expanded significantly since 1968 when the disease was first described, IgA nephropathy is still a common cause of end-stage renal disease. A better understanding of all the factors involved in the pathogenesis of the disease is needed to enable the development of more effective therapies. [ABSTRACT FROM AUTHOR]