Clinicopathological features in six cases of focal fibrocartilaginous dysplasia (FFCD) which involved either the tibia (n = 4) or the femur (n = 2) were reviewed. All cases presented clinical and radiological characteristic features, and histopathological findings were analyzed in five of the six cases. The subject group comprised three boys and three girls, ages ranged from 12 to 18 months. Histologically, the individual lesions showed regional variation in cellularity, amount of fibrous and cartilaginous components. Paucicellular areas were mainly composed of dense fibrous tissue while more cellular areas contained foci of fibrocartilaginous element. The chondrocytes and stellate cells around cartilaginous area were positive for S-100 protein. One case contained both hyaline and fibrocartilage, and architecturally mimicked normal tendinous insertion. One case, which involved proximal tibia, was purely composed of fibrous tissue without fibrocartilage. All cases formed undulating and irregular borders against underlying cortical bone. Histopathologically variable spectrum suggests a strong possibility of undergoing transition from initial cellular and cartilagnous to late paucicellular, fibrous phase. Although any evidence that can explain basic pathogenesis or prognostic histological parameter is lacking, we believe that the term FFCD is not relevant because the presence of fibrocartilage is not an essential feature, and it can cause confusion with other pathological processes. We propose the term 'subperiosteal fibrocartilaginous pseudotumor of long bone' for this unique clinicopathological entity with which heterologous cartilaginous element can be associated.