In a 60-year-old patient with Waldenström macroglobulinemia, a somatic MYD88 L265P variant—present in approximately 97% of cases of this malignancy and known to activate NF-κB signaling—was identified alongside an R264* mutation previously described in the context of immunodeficiency. The gene discussed is NFKB1; the disease is Waldenstrom macroglobulinemia.