OBJECTIVE Burkitt's lymphoma is a unique hematological malignancy remarkable for its biological characteristics, including aberrant expression of the MYC oncogene, and its requirement for intensive treatment regimens. This review will focus on those features, and discuss recent advances in the molecular biology and advancing treatment options for the disease. RESULTS Advances in molecular biology have provided many new insights into the biology and treatment options for Burkitt's lymphoma. Microarray technology has recently been used to define a molecular gene expression signature for Burkitt's lymphoma. This signature allows for the differentiation of Burkitt's lymphoma from other forms of non-Hodgkin's lymphoma such as diffuse large B-cell lymphoma. Recent advances in the use of biological agents, such as rituximab, have also allowed for a reduction in treatment toxicities while still offering comparable survival outcomes for patients. CONCLUSIONS Burkitt's lymphoma is an interesting mature B-cell non-Hodgkin's lymphoma that has numerous distinct features and clinical variants depending on factors such as geographical location, immunological status and patient's age. Although the role of the MYC oncogene has been well studied, we are only now appreciating the defining molecular characteristics of this disease, and using these advances to improve treatment options for patients.