Many hereditary or familial renal disorders have predominantly or only glomerular involvement. This chapter discusses diseases other than Alport’s syndrome and conginetial nephrotic syndrome, which will be discussed elsewhere. Fabry’s disease is also known as Anderson-Fabry’s disease or angiokeratoma corporis diffusum universale. Disease is named after German dermatologist Fabry (1898) and British physician Anderson (1898) who described the skin lesions and other clinical features of the disease, separately at the same time. Aetiopathogenesis is X-linked hereditary disorder of glycosphingolipid metabolism due to deficiency of lysosomal enzyme α galactosidae A, which is essential for the catabolism of ceremide trihexoside, which leads to accumulation of ceremide trihexoside in many organs especially kidneys, vessel wall, skin, nerve, heart, etc. Glycosphingolipid deposition at myocardium, conduction tissues, coronary artery, aorta and valvular leaflets, etc. leads to various arrhythmias and coronary artery disease.