Restrictive cardiomyopathy (RCM) is the least common phenotype of cardiomyopathies with controversy in its exact definition, epidemiology and diagnostic criteria. If left untreated, the disorder could ultimately lead to heart failure. This review article documents available research-based evidence to provide a comprehensive clinical understanding of RCM, its epidemiology, diagnosis and clinical management. The article is organized into ten main sections, namely description, classification, epidemiology, clinical manifestation, prognosis, causes of death, pathophysiology, etiology, differential diagnosis, and clinical management. The article concludes with a concise summary of the main points discussed.