BACKGROUND: Intraosseous lipoma of bone is one of the rarest benign bone tumors, which often involves the metaphysis of long tubular bones, especially the femur, tibia, fibula, and calcaneus. Bone lipoma can be characterized by chronic dull pain but can also be asymptomatic most of the time. As a result, it is less likely to attract people's attention and is occasionally diagnosed through imaging examination during routine physical health check-up. CASE SUMMARY: We describe a clinical case of intraosseous lipoma in a 21-year-old patient with chronic pain in the left lower limb for four years without any significant physical findings apart from the minimal swelling and local tenderness over the median ankle. Computerized tomography suggested the possibility of a lipoma on the left distal tibia, but the pathological examination could make a definite diagnosis. The intraosseous lipoma of the left distal tibia was treated by surgical curettage, bone graft, and internal fixation with steel plate, since the conservative treatment is often ineffective. Postoperatively, the patient made an uneventful recovery and was able to do daily activities without any restrictions. In addition, local recurrence of the intraosseous lipoma was not reported in subsequent reexamination. CONCLUSION: Bone lipoma is very rare and often exhibits no characteristic clinical manifestation. The confirmative diagnosis of lipoma largely relies on a combination of imageology and biopsy. Surgical intervention is often recommended as a conventional therapy for bone lipoma. Postoperatively, the patient makes an uneventful recovery with a good prognosis, and the local recurrence of the tumor is also a low probability event.