This case report presents an exacerbation of NL in the rare setting of a history of glucose-6-phosphate dehydrogenase (G6PD) deficiency and a concurrent diagnosis of Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma (DLBCL). This evidence concerns the gene G6PD and hyperinsulinemic hypoglycemia, familial, 4.