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A case of osteoid osteoma of the elbow in a patient with hemophilia A is described. This male patient presented with chronic and nocturnal pain of the left elbow which was alleviated with acetaminophen. Besides pain, he also complained of stiffness. Before these complaints, he had recurrent bleedings in the elbow because of hemophilia. A delayed diagnosis of osteoid osteoma in the proximal part of the left ulna was established by a bone scan and a multislice spiral computed tomography (CT) scan. The lesion was surgically removed under CT-guidance. The histopathological analyses did not show specific features of osteoid osteoma. Two months after the operation, the complaints decreased and the range of motion of the left elbow improved. A diagnosis of osteoid osteoma of the elbow should be considered in young adult patients with persistent elbow pain and histological confirmation is not always necessary.