Limb Body Wall Complex (LBWC) is a rare heterogenic group of fetal malformations including major defects of the ventral body (thoracoabdominal) wall associated with other anomalies including those of the limbs that may range from amelia to mild positional deformations, unusual craniofacial malformations, and a variety of visceral abnormalities that include the heart, lungs, genitourinary system. Tow phenotypes are described according to the feto-placental attachment. We report a particular case and iconography of LBWC associated with a giant sacral meningocele never described before. The patient was 26 years old, primigravida, with no illnesses or recent infections. She had no history of prenatal exposure to teratogenic agents, nor any family history of congenital malformations. She was referred for polymalformatif syndrome at 30 gestation weeks. Advanced sonography showed giant omphalocel with eviscerated liver, stomac and kidney, club feet, kyphoscoliosis and giant sacral fluid cyst. A 2100 g fetus was delivered, post natal examination confirmed LBWC with abdominoplacental attachment and the particularity of the presence of giant sacral meningocele, two bilateral hemi-genital tubercles and anal atresia.