Burkitt lymphoma (BL) is a very rare non-Hodgkin lymphoma with an extremely short doubling time that tends to affect children in the jaw. Here we report the case of a 6-year-old boy who visited the emergency service complaining of jaw pain, jaw swelling, and dental mobility for 20 days. Biopsy was performed and the diagnosis of BL was established. The patient was immediately admitted at the oncohematology center of a pediatric institute for treatment. Admission examination revealed generalized lymphadenopathy, sagittal sinus thrombosis, and tumor infiltration in the sphenoidal and maxillary sinuses, liver, lung, both kidneys, pancreas, and spinal canal, curving the spinal cord backwards. Treatment started immediately according to the non-Hodgkin lymphoma Berlin-Frankfurt-Munster-95 (NHL-BFM-95) protocol and currently, the patient is in complete remission without signs of disease. This report demonstrates that rapid and precise diagnosis with immediate treatment was essential for therapeutic effectiveness and should be considered for improved BL prognosis.