MicroRNAs are small noncoding RNAs that regulate gene expression at the posttranscriptional level in a sequence-specific manner. After their discovery in 1993, mounting data have provided compelling evidence for their causal involvement in several human diseases, such as cancer and disorders of the brain. MicroRNAs have been described as the main regulator of homeostasis in neurons, and their dysregulation results in pathological conditions in the brain. In this review, we will focus on the role of MicroRNAs as novel drug targets and biomarkers of the three main neurodegenerative disorders (Alzheimer's disease, Parkinson's diseases, and Huntington's disease) and their role in other neurological disorders including traumatic brain injury and status epilepticus.