Background: Hirschsprung’s disease and sigmoid volvulus can sometimes be seen in the same patient.Aims: To investigate the presence of Hirschsprung's disease in patients with sigmoid volvulus and to discuss the diagnosis and treatment methods.Study Design: Systematic review.Methods: This systematic review has been reported in line with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses and the methodological quality of systematic reviews guidelines. The PubMed and Scopus databases were scanned using the keywords “Hirschsprung* volvulus*” and “congenital aganglionic megacolon volvulus*”. The reference list of the selected studies was reviewed for cross-checking. Two reviewers independently screened the available literature. Only the Hirschsprung’s disease cases involving sigmoid volvulus were included, and cases of patients with volvulus in other sites was excluded. There was no restriction with respect to the publication language and type of writing. The primary outcome was morbidity and mortality.Results: A total of 31 cases were analyzed in 22 articles; 97% of the patients were under the age of 40, 90% were male. There was a statistically significant difference in the necessity for relaparotomy between patients who were scheduled for sigmoid volvulus therapy with the suspicion of Hirschsprung’s disease and patients who were treated without suspicion of Hirschsprung’s disease (0% vs 37.5%, p=0.02). While there was no postoperative death in cases with suspected Hirschsprung disease, this mortality rate was 25% in cases without suspicion (p = 0.08).Conclusion: Hirschsprung's disease should be excluded with rectal biopsy if a patient with sigmoid volvulus is under 40 years of age and has complaints of constipation from childhood.