BackgroundHighly aggressive Burkitt lymphoma (BL) with rapidly progressive painful proptosis of the right eye is rarely encountered.Case presentationA 31-year-old Caucasian female presented with rapidly progressive painful proptosis of the right eye and poor visual acuity. Diagnostic Computer tomography, magnetic resonance imagining and angiography, identified an oval-shaped mass on the medial rectus of the right orbit that caused dislocation of eyeball, for which she underwent medial orbitotomy. The mass was histologically confirmed as BL, and postoperative aggressive chemotherapy (BFM-90) was initiated. BFM-90 reduced the recurrence of strabismus, diplopia, and proptosis, but did not correct deficits in the best corrected visual acuity.ConclusionIn patients presenting with painful proptosis and vision loss, a diagnosis of BL should be considered. In addition, because of the rapid progression of BL, and considering that it responds well to treatment, a diagnostic work-up including a tissue biopsy should be initiated immediately if BL is suspected.