Campanacci described this condition in two leg bones, the tibia and fibula,[1] and coined the term.

This condition should be differentiated from nonossifying fibroma and fibrous dysplasia of bone.

Several authors have related this non-neoplastic lesion to adamantinoma – a tumor involving subcutaneous long bones – stating the common cause to be fibrovascular defect.

[4] However, the latter is distinguished from an osteofibrous dysplasia by the presence of soft tissue extension, intramedullary extension, periosteal reaction and presence of hyperchromic epithelial cells under the microscope.

[3] Osteofibrous dysplasia is treated with marginal resection with or without bone grafting, depending on the size of the lesion and the extent of bony involvement.