Neurofibromatosis type 2 (NF2) is an autosomal dominant genetic condition that causes tumorigenicity of bilateral vestibular schwannomas. microRNAs (miRNA) are 22-25 nucleotides single-stranded class of non-coding RNAs that play an essential role in mRNA regulation, mostly negatively, in many human diseases, like cancer. They are able to augment tumorigenicity (as oncomirs) or suppress the process (as tsmiRNAs). Recent studies have elucidated their multifaceted role in transcriptomics of signaling pathways that lead to establishment, development, and invasiveness of tumors. Dysregulated expression level of miRNAs has been reported in various malignancies, as well. In the present review, we aimed to summarize the role of miRNAs in NF2 tumor homing and progression and address their potential in the patients diagnosis, management, and treatment.