Pierre robin sydrome was first reported by Pierre Robin as a triad of mikrognatia, cleft palate, glossopitosis in 1920. It is known that the patient with syndrome have increased risk of a congenital cardiac defects. In this report, we describe a male newborn with cleft palate, mikrognatia glossopitosis and perimembranous ventricular septal defect. Prenatal diagnosis of this syndrome is very difficult. However, postnatal diagnosis can be made simply with a careful physical examination and patients can be scanned with suitable tecnics for anomalies. ©2007, Firat University, Medical Faculty