A 28-year-old Japanese man presented with upper abdominal pain. Computed tomography (CT) revealed a soft tissue mass in the small bowel mesentery. We diagnosed the patient with sclerosing mesenteritis according to the histological findings of small bowel mesentery. Although he was treated with prednisolone, colchicine and azathioprine, neither his symptoms nor CT findings improved. This case is rare in that the disease was refractory. The characteristics of Japanese patients with sclerosing mesenteritis involving small bowel mesentery are not well understood. We herein describe the details of such patients based on a literature review including 32 recently reported Japanese cases.