Case report of a 7 months-child, female, diagnosed with gingival malformation, posterior cleft palate and genitalia hypoplasia. Referred to our institution for investigation of radiological deformity and restriction of extension of the lower limbs in order to perform corrective surgery. PPS is a rare autosomal dominant disease that affects around 1:300.000 newborns. Is an abnormal fibrous tissue in the popliteal region which typically connects the ischium and the calcaneus associated with a number of malformations. May be unilateral or bilateral (usually asymmetrical). The main abnormalities associated with the PPS are facial (cleft palate, cleft lip and fibrous bands in the mouth), genito-urinary tract (cryptorchidism, ambiguous genitalia, hypoplasia) and skeletal (syndactyly, deformities, scoliosis). May be linked to other syndromes (trisomy of 13 or 21, and Turner). The popliteal artery and nerve branches are often abnormal in position, adjacent or in pterygium, and therefore the assessment of the position of these structures is necessary to prevent damage during surgery.