Enterocolitis remains a relatively common complication of Hirschsprung's disease with significant morbidity and mortality. The etiology of Hirschsprung's enterocolitis (HEC) is multifactorial and remains poorly understood. Preventative measures and better treatment modalities will evolve out of a better understanding of the underlying pathophysiology. Prompt recognition of HEC allows early intervention and a potential reduction in disease severity and mortality. This review of HEC describes the epidemiology, clinical and pathological features and current best practice in management. Some of the areas of research into etiology and treatment are discussed.