Thoracoschisis is a very rare congenital anomaly and is frequently found with other congenital defects the of limbs and the abdominal wall as a part of limb-body wall complex (LBWC). Early vascular disruption, amnion rupture and intrinsic embryonic maldevelopment are related to the pathogenesis of this complex. We present a case of thoracoschisis with ipsilateral diaphragmatic hernia. This case had none of the associated anomalies which are seen in LBWC. By ultrasonography performed at 31 weeks' gestation the fetus was misdiagnosed as gastroschisis. In this report we discuss the pathogenetic mechanism of LBWC and our case.