RNA editing is one of the post-transcriptional RNA processes. RNA editing generates RNA and protein diversity in eukaryotes and results in specific amino acid substitutions, deletions, and changes in gene expression levels. Adenosine-to-inosine RNA editing represents the most important class of editing in human and affects function of many genes. The importance of balancing RNA modification levels across time and space is becoming increasingly evident. In this review, we overview the biological significance of RNA editing including RNA editing in tumorigenesis, RNA editing in neuronal tissues, RNA editing as a regulator of gene expression, and RNA editing in dsRNA-mediated gene silencing, which may increase our understanding of RNA biology.