We present an interesting case of a fetus which was delivered in our hospital after antenatal detection on ultrasonography. Clinical examination, ultrasonography findings, infantogram, autopsy and histopathology proved it to be a case of Meckel Gruber syndrome. INTRODUCTION: Meckel Gruber syndrome is a rare, lethal syndrome characterized by occipital cephalocele, post axial polydactyly and dysplastic cystic kidneys. We report a case of Meckel Gruber syndrome diagnosed antenatally at 37 weeks of gestation and confirmed postnatally by postnatal radiographs, fetal autopsy and histopathology. CASE REPORT: A 23 year old primigravida with no history of consanguineous marriage was referred for routine third trimester obstetric ultrasound. US evaluation revealed a single, live fetus of 37 weeks maturity with severe oligohydramnios. US of the fetal abdomen revealed bilateral enlarged echogenic kidneys with few cystic spaces (Fig- 1). Fetal head showed a large occipital cephalocele. (Figure - 2) Fetal thorax and liver were compressed by the enlarged kidneys. However fetal liver did not show cysts. Fetal urinary bladder was visualized. The limbs were examined for polydactyly but could not be ascertained as the liquor was scanty. The lungs were hypoplastic. The umbilical cord had a normal trivascular appearance. As the fetus showed lethal anomalies, she was admitted and delivery was induced. The baby was delivered stillborn. On clinical examination, the face showed characteristic potter facies - micrognathia, hypertelorism, epicanthic folds, snubbed nose, and low set ears. The fetus had grossly distended abdomen, a bell shaped thorax and an occipital encephalocele, lobulated tongue, polydactyly, talipes and club feet (Figures 3, 4, 5). Autopsy was done. The findings included large multicystic kidneys bilaterally. The lungs were hypoplastic. The bladder and ureters were seen and normal, uterus with ovaries was seen. (Figure 6) Histopathologic examination revealed-Multicystic dysplastic kidneys, hypoplastic lungs, and hepatic fibrosis. The finding of polydactyly in the autopsy specimen confirmed the diagnosis of Meckel Gruber syndrome.