Society of Diabetic Nephropathy Prevention Journal of Nephropathology 2251-8363 7 4 2018 10 01 End stage renal disease in a child with oral facial digital syndrome 293 295 10.15171/jnp.2018.58 EN Hossein Emad Momtaz Journal Article 10.15171/jnp.2018.58 2018 05 07 2018 07 10 Background: Oral facial digital syndrome (OFDS) is the name of a group of congenital diseases with involvement of face, oral cavity, digits and other organs. Renal involvement in OFD may be in form of polycystic kidney disease. Case Presentation: This case report presents a patient with OFDS who developed end-stage renal disease (ESRD) at age of six without evidence of renal cyst in sonography. Conclusions: It may be recommended to evaluate and follow up renal function in children with cleft lip and cleft palate especially when they have other anomalies such as polydactyly and oral lesions considering possibility of OFDS.