Burkitt lymphoma (BL) is an aggressive form of B-cell non-Hodgkin lymphoma. It may present with a variety of symptoms leading to possible misdiagnosis and delay in treatment. BL is fatal if left untreated, and early diagnosis and treatment can improve prognosis. In this case report, a 3.5-year-old male patient with no known disease had left eyelid swelling and hematuria, and orbital magnetic resonance imaging performed after his admission showed contrast enhancement in the bulbus oculi, and increased uptake in both kidneys (suvmax:9.5) in positron emission tomography. The patient's bone marrow aspiration was normal. There was no involvement in the evaluation of the central nervous system. As a result of kidney biopsy, he was diagnosed with high-grade B-cell lymphoproliferative disease (Ki-67 95-100%, diffuse positivity with CD79a and EBV). Burkitt lymphoma. The treatment of the patient was started in the NHL-BFM 2012 R4 arm. At the end of the treatment, the ocular findings regressed. Burkitt lymphoma may present with different clinical presentations. If appropriate and rapid imaging techniques are used, positive results on survival can be obtained. Our patient is being followed up alive and well.