Fabry disease is classified as a rare X-linked disorder resulting from complete or partial deficiency of the enzyme alpha-galactosidase A due to mutations in the GLA gene. This genetic disorder leads to the accumulation of globotriaosylceramide (Gb3) within cells, resulting in increased levels of Gb3 in the plasma. The incidence of the disease is estimated at 1:50,000 to 1:117,000 in males. Symptoms of the disease include acroparesthesia (pain in the limbs or throughout the body), hypohidrosis, gastrointestinal problems such as abdominal pain. Various manifestations can appear on the skin, including small red spots around the navel, development of angiokeratomas, and ocular manifestations such as lens opacity and corneal damage. Fabry nephropathy typically begins with microalbuminuria and/or proteinuria, often already in childhood. When patients present with such symptoms, doctors should consider the possibility of Fabry disease through detailed history-taking and physical examination. It is also important to check electrolytes, kidney function, urinary sediment, and perform an EKG and echocardiogram to detect heart problems. Diagnosis is usually confirmed by testing the level of alpha-galactosidase A enzyme, and a skin or kidney biopsy may help if tests are unavailable. Chronic kidney disease is one of the most significant challenges in individuals with Fabry disease, increasing the risk of end-stage kidney disease in this population. Kidney transplantation is the optimal therapy for patients with Fabry disease as recurrence of the disease in the graft is rarely reported and usually occurs many years after transplantation.