This monograph documents in detail the expanding clinical features, recent investigative procedures, a comprehensive history and evolution of the concept of aetiopathogenesis, and a discussion on the differential diagnosis and management of the disease. The whole text has been divided into 7 sections namely, Demography and clinical features, Natural history, Investigative procedures in ICSE, Evolution of the concept of aetiopathogenesis, Differential diagnosis, Management of ICSE and Summary. This monograph will be useful to postgraduate students and also to those who want to have update information on this disease.