Focal fibrocartilaginous dysplasia has previously been described as an
etiology of genu varum deformity in children at about walking age. We
have found 20 cases in the literature, and all of them were located i
n the medial proximal tibial metaphysis. We report five additional cas
es of this entity. The patients had a genu varum deformity, and the an
gulation was at the site of the bone lesion. Four cases were in the ti
bia, and one case was in the distal medial femoral metaphysis. Age at
diagnosis ranged from 13 to 24 months. A cortical defect with surround
ing sclerosis was observed on radiographs. The angulation usually prog
ressed initially, and spontaneous resolution was observed in two cases
at last follow-up. Open biopsy and tibial valgus osteotomy were perfo
rmed in two cases. The unusual femoral case was also treated with open
biopsy and medial hemicircumferential periosteal release. Spontaneous
remodeling of varus angulation and resolution of bony defect may be e
xpected in most cases, and osteotomy can be avoided.