The discovery of microRNAs (miRNAs) as novel regulators of gene expression has led to a marked change in how gene regulation is viewed, with important implications for development and disease. MiRNAs are endogenous, small, noncoding RNAs that largely repress their target mRNAs post-transcriptionally. The regulation of gene expression by miRNAs represents an evolutionarily conserved mechanism that is broadly applicable to most biological processes. Recent studies have begun to define the role of miRNAs in different cell lineages during kidney development, and to implicate specific miRNAs in developmental and pathophysiological processes in the kidney. This review will focus on novel insights into the role(s) of miRNAs in kidney development, and discuss the implications for pediatric renal disease.