High-grade B-cell lymphoma with 11q aberration (HGBL-11q), formerly known as Burkitt-like lymphoma with 11q aberration in WHO-HAEM4R, is an aggressive MYC rearrangement-negative mature B-cell lymphoma with a morphology similar to Burkitt lymphoma (BL) or with an intermediate or blastoid appearance, an immunophenotype (CD10+, BCL6+, BCL2-), and/or gene expression profile (GEP) similar to BL, and a characteristic chromosome 11q-gain/loss pattern. The gene discussed is BCL6; the disease is Burkitt lymphoma.