Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzyme deficiency in the world and renders those affected susceptible to potentially severe oxidative hemolysis. Although the resulting hemolysis is most often associated with drug exposure, it has also been reported after consumption of certain foods. With the exception of review articles that reiterated the historical knowledge that fava beans can provoke severe oxidative hemolysis in individuals with G6PD deficiency, very few articles have examined the safety of other food ingredients and food additives for people with G6PD deficiency. Some articles that associated specific foods with hemolysis appeared to be speculative and based on limited information. The objective of this review was to examine the association between foods, including food additives, and the triggering factors of acute hemolysis. The literature was searched for studies and case reports on food consumption and G6PD deficiency. In this review, fava beans were found to be the only food for which there is conclusive clinical evidence linking the risk of hemolytic anemia to individuals with G6PD deficiency. Food additives, at their permitted level of use in North America, can be consumed safely by most patients with G6PD deficiency.