Retinoblastoma (Rb) is known as one of important childhood malignancies which due to inactivation of the RB gene (tumor suppressor gene in various patients). The early detection of Rb could provide better treatment for Rb patients. Imaging techniques (e.g., MRI and CT) are known as one of effective diagnosis approaches for detection of patients with Rb. It has been shown that utilization of imaging techniques is associated with some limitations. Hence, identification of new diagnosis approaches might provide a better treatment for Rb patients. Identification of new biomarkers could contribute to better understanding of pathogenesis events involved in Rb and provide new insights into design better treatment approaches for these patients. Among the various biomarkers, microRNAs (miRNAs) have emerged as attractive tools for Rb detection. miRNAs are one classes of small non-coding RNAs which could anticipate in a variety of biological process via targeting sequence of cellular and molecular pathways. Deregulations of these molecules are associated with cancerous condition. Multiple lines of evidence indicated that deregulation of various miRNAs involved in various stages of Rb. Here, we summarized a variety of tissue-specific and circulating miRNAs involved in Rb pathogenesis which could be used as diagnostic, prognostic, and therapeutic biomarkers in Rb patients.