Osteoblastomas located on the surface of cortical bone, so-called periostea
l (juxtacortical) osteoblastomas, are extremely rare. A 24-year-old man com
plained of pain and swelling in the left knee. The clinical and radiologica
l investigation showed a tumor located in the posterior portion of the dist
al shaft of the femur. The radiological differential diagnosis included par
osteal osteosarcoma, periosteal chondroma and periostitis ossificans. A fro
zen section was obtained and histology revealed an osteoblastoma with large
epithelioid-appearing osteoblasts consistent with an aggressive osteoblast
oma. An en bloc resection of the tumor was performed and the definitive his
tology of the whole specimen revealed a typical osteoblastoma. The authors
draw attention to the fact that periosteal osteoblastoma is a rare tumor th
at could be mistaken clinically and histologically for other and more commo
n tumors at this location.