Burkitt lymphoma (BL) is a B-cell lymphoma with a germinal center phenotype that has a very rapid doubling time, approximately 24 hours, for which early and accurate diagnosis is critical. There are 3 epidemiologic classifications (endemic, sporadic, and immunodeficiency associated); however, all 3 have indistinguishable histopathology and immunohistochemistry. The role of genetics is critical to detect a translocation of the c-MYC gene. c-MYC gene rearrangements are seen not only in BL but also diffuse large B-cell lymphoma and B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and BL. Treatments for BL have improved drastically during the last decade, primarily with the implementation of pediatric regimens in the adult population. The role of the pathologist is critical in the timely implementation of treatment of adequate intensity. As with many other hematopoietic malignancies, refractoriness to initial therapy is associated with a poor prognosis. This review addresses the pathology, clinical features, histogenesis, and molecular findings of BL and its differential diagnosis.