Introduction: Heart valve disease comprises a cluster of conditions affecting the aortic, mitral, pulmonary and tricuspid valves. This paper reviews all the research on heart valve disease in Malaysia published between the years 2000- 2016. Methods: The methodology was based on the search process described in the paper, “Bibliography of clinical research in Malaysia: methods and brief results”. The search databases included PubMed, Scopus and several Malaysian journals such as MyJurnal and UKM Journal Repository, by using the following keywords: (heart valve disease OR infective endocarditis OR rheumatic heart disease) and (Malaysia). Results: In all 94 papers were identified of which 39 papers were selected and reviewed on the basis of their relevance. The local studies contributed to the knowledge and understanding of the epidemiology, aetiology, pathophysiology, clinical presentations, investigations, treatment, and outcomes of heart valve disease in the country. Discussion: The clinical relevance of the studies performed in the country is discussed along with recommendations for future research.