Purpose <p>Fabry disease is a multisystemic lipidosis where α-galactosidase A gene mutation causes substrate globotriaosylceramide accumulation in the lysosome of the cells. Enzyme replacement therapy is the safest option for treating it. However, in India, it is still available for charitable purposes. Therefore, this study aimed to evaluate the efficacy and limitations of all available treatments in achieving total, complete, and partial remission of Fabry disease.</p> Findings <p>The treatment landscape for Fabry disease has evolved significantly, with enzyme replacement therapy (ERT) emerging as a cornerstone intervention. It transitioned towards halting disease progression from symptom relief, which entails administering α-galactosidase A to restore deficient enzyme activity and mitigate disease manifestations. Despite its benefits, challenges such as neutralizing antibodies and infusion-related reactions persist, underscoring the need for improved therapies. Novel ERT formulations hold promise for enhanced efficacy and reduced dosing frequency for extended plasma half-life but face challenges in tissue distribution. Furthermore, pharmacologic chaperone therapy offers a targeted approach for specific mutations, albeit with limitations in efficacy and patient selection. Substrate reduction therapy aims to decrease substrate accumulation, showing potential in preclinical and clinical studies. However, challenges such as biodistribution and immunogenicity persist. Gene therapy emerges as a promising avenue demonstrating potential in restoring enzyme activity and altering disease trajectory. Additionally, nutritional interventions play a supportive role in managing symptoms and improving treatment outcomes.</p> Implications <p>These findings support the need to develop a more efficient and cost-effective treatment approach. The study underlines the need for research on Fabry disease in India and the need for an indigenous treatment.</p>