The distal radius is an extremely rare site for epithelioid hemangioendothelioma. Joint preservation to maintain a good wrist joint function is rarely reported. The present study described a case of joint preservation surgery in the distal radius with an uncemented 3D-printed endoprosthesis and evaluated the endoprosthesis design and short-term outcomes. A 14-year-old boy was diagnosed with epithelioid hemangioendothelioma in radius. Due to the extensive defect and the excessively short length of the residual distal radius after resection, a custom-made 3D-printed custom-made endoprosthesis was designed and fabricated to reconstruct the defect, with the preservation of the wrist joint. The patient had a favorable wrist function and no endoprosthesis-related complications were observed. The present study presented a case of en bloc tumor resection with joint preservation of the wrist and reconstruction using a 3D-printed endoprosthesis. Satisfactory postoperative function and low complication rates were found.