Fabry disease is a lysosomal storage disorder in which absent or reduced production of alpha-galactosidase A leads to the systemic accumulation of globotriaoslyceramide. Total absence of enzyme production results in the more severe, classic form of Fabry disease, while reduced production of alpha-galactosidase A typically often involves milder symptoms that appear later in life. Episodic pain, angiokeratosis, and hypohydrosis are frequently seen in patients with Fabry disease as well as corneal clouding and hearing loss. Gastrointestinal issues and breathing problems are common, and complications including cardiac abnormalities, kidney disease, and strokes can be life-threatening. Although Fabry disease is an X-linked disorder that primarily affects males, carrier females may become symptomatic. This condition occurs in approximately 1 in 55,000 males, and the development of enzyme replacement therapy has greatly improved the prognosis for many patients.