Encapsulating peritoneal sclerosis (EPS), the most serious and classical complication of peritoneal dialysis (PD), was recognized soon after the introduction of PD. Within a little more than a decade after PD was first introduced in Japan, the country began experiencing an increasing number of EPS cases. The present paper discusses the clinical stages of EPS, the incidence of EPS in Japan and the as-yet incompletely understood mechanisms of its development, and EPS management and prevention.