Background: Giant cell tumor is a benign bone tumor, but can be malignant, metastatic or recurrent. Giant cell tumors of the distal humerus are extremely rare. Case presentation: A 57 years old male patient with Campanacci grade III giant cell tumor of the distal humerus who underwent en bloc resection of the tumor and cemented “sloppy-hinged” total elbow arthroplasty. Preoperative Mayo Elbow Performance Score was 25. At the 6-month follow-up, Mayo Elbow Performance Score was 90, the movement has improved significantly with no pain. There were no signs of local recurrence and metastasis. Conclusion: The en bloc resection of the tumor followed by total elbow arthroplasty surgery has very good results in pain relief and rehabilitation. However, the patient should be monitored further to detect recurrence and metastasis as well as complications related to the elbow components.