A five-year-old boy presented with left tibial swelling; there was a typical radiological feature of osteofibrous dysplasia. He was observed until the age of 14 years; there was development of aggressive radiological features. Biopsy revealed chondroblastic osteosarcoma. It was excised and the histology revealed chondroblastic osteosarcoma. A small lesion distal to the main tumour revealed adamantinoma within a background of feature of osteofibrous dysplasia. Proximally, feature of osteofibrous dysplasia was identified focally. The finding of adamantinoma over the distal part of the lesion supports that feature of osteofibrous dysplasia may be a precursor of adamantinoma. They may be a spectrum of diseases with multistep neoplastic transformation. The osteosarcoma may be a result of de-differentiation from adamantinoma. This case is remarkable as the patient was only 14 years old and the tumour showed typical features of osteosarcoma, with feature of osteofibrous dysplasia and adamantinoma found in the same specimen. Feature of osteofibrous dysplasia may be a precursor of adamantinoma, and adamantinoma may dedifferentiate into osteosarcoma.