A class of illnesses known as lysosomal storage disorders is brought on by deficiencies in membrane transporters, lysosomal enzymes, or other proteins important in lysosomal biology. The diseases are categorized based on the kind of material that builds up; lipid storage disorders, glycoproteinoses, and mucopolysaccharidoses are a few examples of this. In cases of mucopolysaccharidoses, glycosphingolipidoses, and lysosomal glycogen storage diseases, cardiac disease is particularly significant. Valvular disorders and hypertrophic and dilated cardiomyopathy could be seen. Endomyocardial biopsies are crucial for making diagnoses. The diagnosis of these illnesses is aided by microscopic characteristics as well as auxiliary procedures such as ultrastructural studies and specific stains. Further confirmation of the diagnosis is obtained through enzymatic and molecular genetic investigation. According to new research, enzyme replacement therapy significantly improves the disease's characteristics, including cardiac involvement. Due to the availability of ERT, the requirement for family screening, and the importance of proper patient management and counseling, it is critical to identify these illnesses.