An original case is reported: spinal cord compression due to extramedullary hematopoiesis of the epidural space, related to Paget's disease of bone, successfully treated by surgery. Extramedullary hematopoiesis, usually occurring in cases of hematological disease, can also complicate Paget's disease, probably by bone effraction mechanism. A review of literature has found 5 other cases of extramedullary hematopoiesis occurring in Paget's disease, without hematological disease. Medullar scintigraphy (Indium111, Technetium99 Sulfur colloid) can confirm the diagnosis after X-ray findings, without the need for biopsy. Awareness knowledge of this complication can sometimes avoid invasive investigation. Low dose radiotherapy is useful in the case of hematological disorders. In Paget's disease, there is a risk of sarcomatous degeneration. In our patient, surgery cured cord compression, but it relapsed because of extensive fibrosis, in complicating surgical hemorrhage.