Abstract. Desmoplastic fibroma of the bone is an extremely rare primary benign tumor. The present study reports a case of desmoplastic fibroma of the bone with the longest published follow-up. A 21-year-old female presented to The First Hospital of Jilin University (Changchun, Jilin, China) with thigh pain. Radiography demonstrated a lytic expansile lesion in the proximal femur. Curettage was performed, followed by use of an allogeneic graft. One month later, the patient suffered a pathological fracture and was treated with an open reduction and internal fixation. There was no recurrence of the tumor over a 28-year follow-up period. In conclusion, desmoplastic fibroma in the proximal femur is rare and an intralesional resection is strongly recommended to prevent recurrence. The disease may be misdiagnosed as a bone cyst, so the diagnosis should be confirmed with a histological examination.