Background: Cystic echinococcosis is a worldwide disease caused by Echinococcus granulosus. The human leukocyte antigen (HLA) class II has been associated with susceptibility or resistance to this disease. Aim: To systematically review the studies that reported the association of different HLA class II alleles with susceptibility or resistance to cystic echinococcosis. Methods: Scientific papers were searched in PubMed (HLA AND Hydatidosis, HLA AND Echinococcus granulosus, Cystic echinococcosis AND HLA) and in Google Scholar (HLA + hidatidosis, HLA + equinococosis quística, HLA + Echinococcus granulosus, HLA allele frequencies + cystic echinococcosis). Scientific papers were selected according to inclusion and exclusion criteria. Results: 1176 results were obtained, and seven articles published between 2004 and 2018 were included in the systematic review, with samples of patients from the Middle East and North Africa and Latvia. The DR1, DRB1*0403, DR8, DR10, DR52 and DQA1*0101 alleles were associated with resistance against cystic echinococcosis, while the DR3, DRB1*0401, DR7, DR11, DR16, DRB1*1701, DQB1*0302 and DQA1*0401 were associated with susceptibility. The presence of DR15 could be associated with resistance or susceptibility. Conclusion: A larger number of studies with different populations are needed to identify whether the results synthesized in this review are representative of other regions.