Limb body wall syndrome (LBW) is one of the rarest malformation complexes. It is also known by other names as « Abnormal stem of the body » « Congenital absence of the umbilical cord » and « cyllosome and Pleurosomus » [1]. This complex is a heterogeneous disease characterized by multiple severe congenital abnormalities of the fetus with exencephalon/thoraco-encephalo and/or abdominoschisis (anterior parietal malformations) and malformations of the limbs, with or without facial slits. The diagnosis remains difficult to establish in view of the clinical polymorphism the prenatal ultrasound can detect this abnormality during the first trimester. The exact etiology of this condition is still uncertain, the theory of amniotic bands of Tropin and the vascular theory (early vascular accident) of Van Allen have not succeeded in explaining all the abnormalities observed in the LBWC [2]. Karyotype study are normal and there has been no evidence of correlation with sex, age of parents or teratogenic agents. Prognosis is fatal death occurring in antenatal or early neonatal period. We are describing two cases of Limb body wall syndrome (LBW).