Fabry disease is a rare, X-linked lysosomal storage disorder, caused by an inborn deficiency of alpha-galactosidase A. The resulting inability to catabolize glycosphingolipids causes progressive accumulation of globotriasylceramide (Gb3). The resulting symptoms usually appear during childhood and adolescence, followed by disease progression and premature death. The disease manifests primarily in affected homizygous males and to some extent in heterozygous (carrier) females with a mild or severe degree because of random X-chromosomal inactivation.