Uveal melanoma (UM) is the most common and aggressive primary intraocular tumor in adults. UM is classified as a malignant tumor with a strong tendency of metastasis, which always leads to poor outcomes. At present, the pathogenesis of UM remains unclear and lacks effective therapies. Recent studies have shown that microRNAs (miRNAs), defined as a group of 21-23 nucleotides single-stranded noncoding RNAs, play a significant role in UM. By binding to the complementary sites within the 3' untranslated region (3'UTR) of message RNAs (mRNAs), miRNAs regulate genes by decaying mRNAs or inhibiting their translation. Thus, miRNAs can modulate various biological behaviors of tumors, including cell proliferation, invasion and metastasis. Furthermore, miRNAs have shown clinical applications by serving as biomarkers for diagnosis and prognosis, regulating immune response, and functioning as epigenetic regulators. It is reasonable to believe that miRNAs have wide application prospects in the early diagnosis and therapy of UM.