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Abstract
A 41-year-old Caucasian woman presented with a painful, red right eye with minimal systemic symptomatology, and was initially diagnosed with right idiopathic orbital inflammation. Ten days later, she developed abdominal and respiratory symptoms; this led to her demise within a further week. Post-mortem examination demonstrated widespread extranodal NK/T- cell lymphoma (nasal type), involving the right posterior orbit, lungs, uterus, left adrenal gland, pericardium and meninges. Thorough physical examination with early orbital biopsy should be considered to exclude underlying treatable pathology in managing patients with presumed idiopathic orbital inflammation.