Although the usefulness of vascularized fibular grafts has been well documented, periarticular reconstruction remains challenging. We report a case in which a folded vascularized fibula was successfully used for reconstruction after resection of a locally aggressive bone tumor in the distal humerus. A 56-year-old man had polyostotic fibrous dysplasia in his right extremity, and underwent multiple surgeries on right elbow until the age of 44 years. After a 12-year asymptomatic period, he developed severe pain in his right elbow. Imaging studies revealed extensive osteolytic changes and pathological fracture in the distal humerus. Under the diagnosis of secondary aneurysmal bone cyst, two-stage surgery was performed. First, tumor resection combined with a cement spacer and external fixation was performed. Five weeks after the first surgery, reconstruction using the vascularized fibula was performed. After harvesting the graft, it was divided into two segments, which were then placed in parallel into the distal humerus. Bone union was observed 3 months after the second surgery. Although the range of motion was limited, the patient was satisfied with the functional outcome. Thus, folded vascularized fibula may be the procedure of choice for reconstruction of massive defects in the distal humerus.