Introduction: Holoprosencephaly (HPE) is a rare congenital anomaly due to incomplete or absent division of the prosencephalon or forebrain into distinct cerebral hemispheres. Case Report: A near term baby boy, born at 37 weeks of gestation to a 30 year old mother, in peripheral hospital without antenatal investigation like ultrasonography was admitted to our hospital with provisional diagnosis of anticipated sepsis. Cranial sonography of the patient revealed absence of septum pellucidum with dysplastic corpus callosum, partially formed frontal horns of lateral ventricles, colpocephaly and azygous anterior cerebral artery diagnosed as lobar holoprosencephaly. MRI brain confirmed all the features of cranial sonography with additional depiction of fused cingulated gyrus. Conclusion: Holoprosencephaly is a rare congenital structural anomaly of the prosencephalon that results in incomplete development of the brain. In its severe form it is incompatible with life. It is prudent to diagnose holoprosencephaly prenatally and determine the type to classify severity, complications and survival rate.