BACKGROUND Primary hydatid disease in the colon is an extremely rare finding. We performed a systematic review of cases of hydatidosis of the colon published to date. AIM To systematic review the published papers about hydatidosis of the colon. METHODS Following PRISMA guidelines, we performed an unlimited search for articles on CH in the MEDLINE (PubMed), EMBASE, Cochrane, Latindex and Scielo databases, published in English, Spanish, French, German or Italian between January 1, 1960 and December 15, 2018. The search items were: (Colon) AND [(hydatidosis) OR (hydatid disease) OR (echinococcosis)]. RESULTS Nine case reports were found [five men, four women, median age 64.55 years (range: 21-81)]. The most frequent clinical manifestation was abdominal pain (8/9) (88.9%). In almost all cases several image studies were carried out, but abdominal computed tomography was the most used (66.7%). Five resections of the colon (62.5%) and three sutures of the fistula between cyst and colon (37.5%) were performed. Four patients did not present post-operative complications, but one died. Postoperative period was recorded for a mean period of 35 months. No recurrences were recorded. CONCLUSION The most frequent symptom was abdominal pain (evidence level 4). Computed tomography was the most frequently used diagnostic imaging technique (evidence level 4). Colectomy was the most appropriate treatment (evidence level 5). All the diagnostic and therapeutic options for hydatid cysts located in colon have a low level of evidence.