Background Cholangiohydatidosis (CH) is an evolutionary complication of hepatic cystic echinococcosis, associated with increased morbidity and mortality. The aim of this study was to describe the available evidence regarding clinical characteristics of CH, postoperative complications and hospital mortality. Methodology/Principal findings Systematic review. Studies related to CH with no language or publication restriction were included. Sensitive searches were performed in Trip Database, SciELO, BIREME-BVS, WoS, PubMed, EMBASE and SCOPUS. MeSH and free terms were used, including articles up to April 2023. The main outcome variables were postoperative complications and hospital mortality; the secondary ones were publication year, origin and design of primary studies, main clinical manifestation, anatomical location and type of cysts, hospital stay, surgical procedure performed, reinterventions; and methodological quality of primary studies, which was assessed using MInCir-T and MInCir-P scales. Descriptive statistics, calculation of weighted averages and their comparison by least squares logistic regression were applied. 446 studies were retrieved from the searches performed, 102 of which met the inclusion and exclusion criteria. The studies analyzed represent 1241 patients. The highest proportion of articles was published in the last decade (39.2%). Reports are mainly from Turkey (28.4%), Greece (9.8%), Morocco and Spain (8.8% each). With a weighted mean of 14.3 days of hospital stance; it was verified that 26.2% of patients developed postoperative complications (74,3% Clavien y Dindo III y IV), 6.7% needed re-interventions, and 3.7% died. When comparing the variables age, postoperative complications, hospital mortality, and reinterventions in two periods of time (1982–2006 vs. 2007–2023), no statistically significant differences were found. When applying the MInCir-T and MInCir-P scales, the methodological quality of the primary studies was 9.6±1.1 and 14.5±4.3 points, respectively. Conclusion/Significance CH is associated with severe postoperative complications and significant hospital mortality, independent of the development of therapeutic support associated with the passage of time.