Abstract: Encapsulating peritoneal sclerosis (EPS) is a rare but devastating complication with high mortality rate in long- term peritoneal dialysis patients. It is characterized by progressive and extensive fibrotic thickening and sclerosis of the peritoneum, leading to encapsulation of bowel and intestinal obstruction, but is hard to be recognized at its early stage. The pathogenesis of EPS remains elusive. No specific treatment has been developed, and a multidisciplinary combination therapy is adopted at the present time. In this paper, we introduce the pathogenesis, diagnosis and treatment of EPS.