Focal fibrocartilaginous dysplasia is a rare benign entity, previously described as a factor responsible for causing tibia vara at the walking age. We report here the case of a child with tibia valga due to focal fibrocartilaginous dysplasia of the lateral part of the proximal tibia. To our knowledge, this is the first observation of a lesion located in the lateral proximal tibia. Clinical, radiographic and magnetic resonance imaging surveillance was performed. Spontaneous resolution of the lesion with correction of the angular deformity did occur.