Introduction: Burkitt lymphoma (BL) is rare, but an aggressive malignancy. Central nervous system Burkitt lymphoma (CNSBL) is very rare. CNS Burkitt’s lymphoma is treated with high-dose chemotherapy and radiation. Radiotherapy in children younger than three years old may cause major CNS damage.

Case report: A three-year-old boy presented with painful proptosis of the left eye. He underwent adenoidectomy 2 months before admitted to our center. Sleep difficulty was not resolved after surgery. Microscopic study of paranasal sinus biopsy and IHC confirmed the diagnosis of Burkitt Lymphoma.

Conclusions: Inany patients presenting with painful proptosis, BL should be considered. Adenoid hyperplasia should not be considered as a sole cause of sleep difficulty.