Fabry disease (FD) is an X-linked disorder of glycosphingolipid catabolism, which leads to the gradual accumulation of the enzyme's principle substrate, globotriaosylceramide; this results in the progressive damage of renal epithelial cells, cardiac myocytes, vascular endothelium, and neurons of the dorsal root ganglia and autonomic nervous system. Diagnosing patients with FD is challenged by variable clinical presentation, lack of pathognomonic features, and considerable symptom overlap with more prevalent conditions. With advances in molecular biology, there is now a better understanding of FD, including its prevalence, mechanisms of inheritance, genotype-phenotype relationships, and the impact of the disease in female carriers. These discoveries, along with the advent of efficacious, disease-specific therapies offer the potential to significantly improve the identification and care of patients living with FD. This archived in-office rounding activity will examine advances in our understanding of FD pathophysiology and genetics, including unique considerations in heterozygous female patients. It will also review treatment options for patients with FD and discuss strategies for nephrologists for developing patient-specific management plans.