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Hemoglobin E (HbE) beta-thalassemia is common in Asian countries. Extramedullary hematopoiesis (EMH) is a chronic complication of this condition, which is a rare cause of paraplegia. Here, we discuss an uncommon presentation of HbE beta-thalassemia with EMH. A 32-year-old male patient presented with spastic paraplegia at C7 level. Contrast magnetic resonance imaging showed epidural extramedullary soft-tissue clumps at D2–D7 vertebra. Histology section showed numerous pleomorphic large cells admixed with colonies of small cells having rounded contour. A relook at the history revealed a history of hemolytic anemia diagnosed at the age of 12 years, for which he was on 1–2 transfusions/year. HbE beta-thalassemia had been diagnosed 20 years before. Hence, a final diagnosis of EMH was made. Although such case reports have been documented, the amount of available data is limited. A high index of suspicion for EMH should be held in patients with hematological disease who present with neurological disorders.