A 14 year old boy with proximal focal femoral deficiency (PFFD) on left side, contralateral hip dysplasia in association with ulnar hypoplasia and cleft hand was seen our clinic one year ago. From our research, despite it is atypical presentation, this case in a broad context conforms with the femoral-fibula-ulna complex (FFU). This boy was born to healthy and nonconsanguineous parents. Pregnancy and delivery were uncomplicated, and no history of prenatal teratogen exposure, any drug ingestion, infection, diabetes mellitus or other conditions that could be associated with malformation. The patient refused any sort of surgical treatment.