Restrictive cardiomyopathy is a myocardial disorder characterized by restrictive ventricular filling. It represents one of the three phenotypes originally classified by the World Health Organization besides dilated and hypertrophic cardiomyopathies. Of the three, it is the least common phenotype but has an ominous prognosis with the highest recorded rates of sudden cardiac death. Clinical trials investigating RCM have had varied findings. This is attributable to the current classification systems of cardiomyopathy based on morphological and structural alterations rather than etiologic-based. The classification has created etiological heterogeneity requiring a combination of methods for a confirmatory diagnosis as well as complicating clinical management methods, which lacks standardized guidelines and largely relies on the standard heart failure therapy. Thus, the present review discussed current research evidence to produce a comprehensive understanding of the clinical status of RCM, including its diagnosis and clinical management.