IgA nephropathy is globally the most common primary glomerulonephritis, but the pathogenesis of this condition is still only partially understood. MicroRNAs (miRNAs) are short, noncoding RNA molecules that regulate gene expression. Genome-wide analysis of renal miRNA expression has identified a number of novel miRNAs related to immunological and pathological changes. Specifically, overexpression of miR-148b might explain the aberrant glycosylation of IgA1, which has a central pathogenetic role in the early phase of IgA nephropathy. By contrast, miR-29c is an antifibrotic miRNA that is probably important in the late stages of disease progression. In addition, urinary levels of several miRNAs are significantly changed in patients with IgA nephropathy compared with healthy individuals; some alterations seem to be disease-specific, whereas others are apparently damage-related. As miRNAs in urinary sediment are relatively stable and easily quantified, they have the potential to be used as biomarkers for the diagnosis and monitoring of disease. However, to date, limited data are available on the role of miRNAs in the pathogenesis of IgA nephropathy and their potential application as biomarkers. Consequently, further studies are urgently needed to address this shortfall. Here, we review the available literature on miRNAs in relation to IgA nephropathy.