Acute kidney injury (AKI) is common and predicts mortality in severe malaria. Studies have reported an increased incidence of AKI in males with hemolytic features of SM. G6PD deficiency, an X-linked enzymopathy, can induce hemolysis. We evaluated whether the G6PD African allele (A-) was associated with AKI in children with severe malaria. The prevalence of G6PD deficiency was 16.7% among hemizygous males and 2.4% in females. G6PD deficiency was associated with 2.56-fold odds of AKI (95% CI, 1.33 to 4.93, p=0.005), adjusting for age, sex, site, nutritional status, and features of hemolysis.