Address for Correspondence: Dr Sukhinder Baidwan, House No 313, Sector 71, Mohali, Sas Nagar, Punjab-160071, India. Tele No.: +919872812002 E-Mail: dr.sukhi313@gmail.com Access this Article online Quick Response code Web site: 1,3 Professor, Department of Anatomy, Gian Sagar Medical College & Hospital, Ramnagar, Banur, Patiala, Punjab, India. *2 Associate professor, Department of Anatomy, Gian Sagar Medical College & Hospital, Ramnagar, Banur, Patiala, Punjab, India. Iniencephaly is a rare neural tube defect (1 in 65,000 births in India). It involves defect of occiput and inions combined with rachischisis and retroflexion of head. We present a rare case of inencephaly diagnosed on ante-natal ultrasound in a 13 weeks fetus and associated with cystic hygroma and single umbilical artery. The fetal cervico-thoracic spine was retroflexed, occipital area was soft and brain tissue and spinal cord was visible externally in cervical region. The side of neck showed subcutaneous edema and two vessels (1 umbilical vein and 1 umbilical artery) were present in the umbilical cord. On x-ray examination spinous processes of all vertebrae showed presence of ossification centers except in the cervical region.