BACKGROUND Aggressive giant cell tumor of the distal fibula is so rare that no consensus on a surgical strategy has been reached. Thus, an appropriate treatment strategy is still important to discuss. CASE SUMMARY A 61-year-old man who had been experiencing progressive swelling of the left lateral malleolus accompanied by pain for half a year was presented at our hospital. He had never been treated prior to coming to our hospital. Preoperative imaging revealed a 10 cm × 6 cm mass located in the body of the distal fibula. Pathological biopsies confirmed it was a giant cell tumor. Preoperative examination revealed he had dilated cardiomyopathy with class 3 cardiac function. The cardiologist and anesthesiologist determined that he could tolerate the operation, but the operation should be as short and minimally invasive as possible. With the patient’s consent, we performed a tibiotalar fusion and followed up with him for 2 years, finding no recurrence and a satisfactory recovery. CONCLUSION Tibial talus fusion is an effective method for the treatment of distal fibula tumors.