Background: Anencephaly and spina bifida are the commonest type of Neural tube defect. Exencephaly is the severe form of anencephaly in whichthere is not only deficiency of scalp and cranial vault but also uncovered immature braintissue protrudes out of the cranium. There isloss of fetal brain tissue to variable degrees and it is considered a precursor to anencephaly . Most cases are stillborn. Present study was carried outtostudy exencephaly and the associated morphological abnormalities. This study also compares the findings with other studies and defines a clinical entity that is incompatible with human life. Method: The study was carried out on a collection of 20 aborted fetuses in department of anatomy out of which 11 showed presence of exencephaly. Result: These exencephalic fetuses presented with facial abnormalities, cvs abnormalities, skeletal deformities and abdominal abnormalities.This study describes in detail the severe form of NTD called exencephaly and the associated abnormalities. We have described a lesser defined condition that is exencephaly, the anatomical knowledge of such may be important for academic, clinical as well as radiological procedures .With  the detailed knowledge of the condition the diagnosis ofexencephaly, can be established sonographically even in the first trimester and necessary measures can be taken.