Craniorachischisis, a lethal congenital malformation, is an extremely rare and severe type of open neural tube defects arising from defective morphogenesis of central nervous system during embryonic stage of life resulting in fissure of the skull and vertebral column. We report the case of a newborn female baby exhibiting features of craniorachischisis, which was detected after birth following assisted breech vaginal delivery of a 36year old woman who presented to us at 30 weeks of gestational age, in second stage of labor. Through this case report, our objective is to reemphasize that interventions like health education, parental counselling, use of folic acid along with additional micronutrients in peri–conceptional period and throughout the pregnancy are crucial, especially in low resource settings, substantially decreasing the risk of neural tube defects.