MicroRNAS (miRNAs) are a class of endogenously single-stranded non-coding RNA molecules that can negatively modulate the expression of target messenger RNAs by 3´ UTR base pairing. During the processing of a miRNA, a network of orchestrated molecular events provides a dynamic manner to posttranscriptionally modulate gene expression. Recent research has demonstrated that although these molecules are small, they are involved in several crucial biological functions, as well as, in a broad spectrum of human diseases. In this review, we highlighted the current knowledge on the miRNA pathway field, focusing on how the disruption of the miRNA-mediated silencing pathways could lead to the pathogenesis of neurological disorders. The potential use of miRNAs as diagnostic/prognostic markers and the possibility of reversing the effects of some miRNA polymorphisms/mutations by promising therapeutics procedures have brought new perspectives into the treatment of human pathologies.