Human Genome Project has made it clear that a majority of the genome is transcribed into the non-coding RNAs including microRNAs as well as long non-coding RNAs (lncRNAs) which both can affect different features of cells. LncRNAs are long heterogenous RNAs that regulate gene expression and a variety of signaling pathways involved in cellular homeostasis and development. Studies over the past decade have shown that lncRNAs have a major role in the kidney pathogenesis. The effective roles of lncRNAs have been recognized in renal ischemia, injury, inflammation, fibrosis, glomerular diseases, renal transplantation, and renal cell carcinoma. The present review outlines the role and function of lncRNAs in kidney pathogenesis as novel essential regulators. Molecular mechanism insights into the functions of lncRNAs in kidney pathophysiological processes may contribute to effective future therapeutics.