Burkitt lymphoma (BL) is a type of highly invasive non-Hodgkin’s lymphoma and one of the most rapidly growing malignant tumors. The etiology and pathogenesis of BL have not been fully elucidated. Previous studies have shown that BL may be related to c-myc translocation. The etiology of BL has been thoroughly studied in recent years, and some new tumorigenic mechanisms have been found in the field of gene mutation and epigenetic regulation. The aim of this review is to elucidate the pathogenesis of BL from the perspective of gene mutation and epigenetic regulation.