Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations in the GLA gene that result in deficiency of enzyme α-galactosidase A activity. Clinical manifestation varies from mild to severe depending on the phenotype. The main clinical manifestations are cutaneous (angiokeratomas), neurologic (acroparesthesias), gastrointestinal (nausea, diarrhea, and abdominal pain), renal (proteinuria and kidney failure), cardiovascular (cardiomyopathy and arrhythmias), and cerebrovascular (stroke). Enzyme replacement therapy with recombinant human α-galactosidase is currently the therapeutic option for FD. Although enzyme replacement therapy has changed the natural history of disease, many clinical aspects of FD require an additional specific treatment. Nutritional approach is mostly indicated in case of nephropathy and gastrointestinal symptoms. Specific dietary interventions can modulate some pathogenetic mechanisms of the disease, such as the inflammation, oxidative stress, and autophagic disorders. However, to our knowledge, limited attention has been given to the nutritional aspects of FD. The aim of this review is to examine nutritional strategies that might interfere with several pathophysiologic aspects of FD, including inflammation and oxidative stress. A dietary approach should be part of the basic treatment in renal manifestations of FD. Dietary measures recommended for irritable bowel syndrome could be recommended for gastrointestinal symptoms. Dietary factors can modulate the inflammation, oxidative stress, and autophagy involved in FD. Polyphenols, ω-3 fatty acids, microbiota, and specific dietary patterns can interfere with inflammation/oxidative stress and autophagy mechanisms and could also contribute to the slowing of FD progression.Copyright © 2019 Elsevier Inc. All rights reserved.