Based on the paradoxical observation that EBV-LPD frequency is increased in ATM patients while they do not exhibit major T-cell deficiency, we raise the hypothesis that the defect of ATM in EBV-infected cells could play a role per se in the control of EBV latency, favoring a latent program more prone to lymphomagenesis. The gene discussed is ATM; the disease is disseminated peritoneal leiomyomatosis.