Non-Hodgkin lymphoma (NHL) accounts for 10% of pediatric malignancies and is the third most common malignancy in childhood. We report a previous healthy 3-year-old boy with stage IV Burkitt lymphoma presenting with acute visual loss. In funduscopy his pupils were fixed at 6 mm in diameter without light perception. He started therapy according to the UKCCSG 9003 protocol. The boy didn't show any visual improvement but some slight light perception despite his partial remission after CYVE II. He died of neutropenic septicemia before his scheduled radiotherapy. In conclusion, loss of vision is a rare presenting sign in NHL. Differential diagnosis is crucial as early diagnosis and effective treatment are important for survival as well as for visual restoration.