Background: Hirschsprung's disease is characterized by the absence of enteric ganglia in the distal digestive tract; being usually diagnosed in neonates, it rarely debuts during adulthood, where it presents as chronic constipation refractory to treatment. Case Report: 24-year-old female patient with Hirschsprung's disease who arrives to the emergency room with a sigmoid volvulus requiring urgent surgical therapy. Conclusions: Hirschsprung's disease doesn't frequently debut in adulthood, presenting a diagnostic and therapeutic challenge for the general surgeon, especially when dealing with its complications.