Agnathia, holoprosencephaly and situs inversus complex is an extremely rare form 
of congenital malformation. Though a few cases have been reported from other 
parts of the world, to the best of our knowledge none has been reported from 
India so far. Maternal alcoholism is regarded as an important factor causing 
holoprosencephaly. Disruption of the Shh gene signaling pathway is also said to 
be a factor for the occurrence of holoprosencephaly as well as left right 
asymmetry. Though several factors are suspected as a cause of this deformity, 
the precise aetiopathogenesis is still under debate. Lack of knowledge might be 
due to paucity of data from cases due to its rarity. Hereby, we are presenting a 
case of agnathia, holoprosencephaly and situs inversus born at 32 wk of 
gestation by an alcoholic mother. Externally the child had agnathia and 
cyclopia. There was no mandible or any oral cavity. It was accompanied by 
noticeable limb deformity. Internally there was holoprosencephaly, situs 
inversus totalis with several visceral abnormalities. To the best of our 
knowledge this is the first case of agnathia, holoprosencephaly and situs 
inversus complex to be reported in an indexed literature from India. This report 
also strengthens the association of maternal alcoholism with occurrence of 
holoprosencephaly.