This comprehensive review explores the pathogenesis, clinical features, pathology, and diagnosis of Burkitt Lymphoma (BL). BL arises from B-cells within the germinal center and is driven by the constitutive expression of the MYC proto-oncogene on chromosome 8q24, leading to deregulation of cellular processes like cell growth, division, and apoptosis. The characteristic chromosomal translocation involving MYC is a result of errors during immunoglobulin gene diversication in B-cells. Additional mutations complement MYC deregulation. EBV infection is implicated in African BL cases and some sporadic and immunodeciency-associated cases. Clinically, BL presents with rapid tumor growth and spontaneous tumor lysis, resulting in elevated LDH and uric acid levels. The three clinical forms are endemic, sporadic, and immunodeciency-associated, each with distinct characteristics. Accurate diagnosis relies on anatomopathological evaluation and identication of MYC translocations. Treatment involves intensive chemotherapy with CNS prophylaxis, and rituximab has been shown to improve outcomes. This review emphasizes the need for precise diagnosis and tailored management to combat this aggressive malignancy effectively.