While previous descriptions of patients with CRB2 disruption lack reports of infectious or immunologic complications or data on transplant outcomes, a documented case of B cell lymphoma in a young child—resulting in death at 3 years of age due to chemotherapy complications [8]—raises suspicion of a potential link between the underlying genetic defect and immune dysregulation. The gene discussed is CRB2; the disease is B-cell non-Hodgkin lymphoma.