Background <p>MicroRNAs (miRNAs) are a subclass of small non-coding RNAs that regulate gene expression and play important roles in infectious diseases. Echinococcosis is a globally distributed disease caused by various species of <i>Echinococcus</i>. The disease can be asymptomatic in the early stages, but if left untreated, it can lead to severe complications. Like other helminths, <i>Echinococcus</i> spp. exhibit immunomodulatory capacities that favour infection establishment and progression. Currently, there is no fully accurate, non-invasive method for early detection of echinococcosis. This systematic review provides a comprehensive analysis of the existing literature and discusses recent advances in the study of miRNAs during host–<i>Echinococcus</i> spp. interactions, emphasizing their intriguing role in immunomodulation and their potential as biomarkers for detection, disease activity, prognosis, and therapeutic monitoring, as well as their possible relevance for future therapeutic research.</p> Methods <p>This systematic review was conducted in accordance with the PRISMA 2020 guidelines. Searches were performed in PubMed, Web of Science, and Scopus without date restrictions to identify studies assessing the diagnostic potential of miRNAs in cystic echinococcosis (CE) and alveolar echinococcosis (AE). Fourteen original research articles were included in the final analysis.</p> Results <p>Parasite-derived miRNAs have been detected in plasma, serum, and liver tissue, with egr-let-7-5p and egr-miR-71-5p emerging as candidates for CE detection and monitoring. In CE, dysregulation of host miRNAs was observed, displaying cyst activity-dependent patterns associated with inflammation, immunomodulation, and fibrosis, including a consistent upregulation of hsa-miR-146a-5p in plasma and serum samples. In AE, several host miRNAs exhibited altered expression in liver tissue and plasma, with hsa-miR-483-3p consistently upregulated. Comparative analyses revealed partially distinct but overlapping miRNA profiles in CE and AE, reflecting host-driven regulatory responses related to immunomodulation and tissue remodeling with stage-dependent patterns. Most included studies were based on case–control designs, with small sample sizes and limited validation, suggesting high overall risk of bias.</p> Conclusions <p>Current evidence suggests that miRNAs have translational potential as complementary biomarkers for diagnosis, patient stratification, prognostic assessment, and disease monitoring in echinococcosis and may support future therapeutic research. Nonetheless, methodological heterogeneity and limited validation may contribute to overestimating diagnostic performance and hinder clinical translation.</p> Graphical Abstract <p></p>