Holt- Oram Syndrome: Is a genetically determined Autosomal dominant disorder with 100% penetrance and variable expression in which aplasia or hypoplasia of digital bone present and associated with congenital heart diseases.

METHODS : A 28 years old (62Kgs, Ht-152Cms) case of G2P1L1 with post caesarian pregnancy with Term Gestation came to our Tertiary care teaching hospital and private medical college in costal Andhra Pradesh, India in the month of July 2014 for safe confinement.

RESULTS : Left upper limb fore arm relatively shorter than right upper limb and there is absence of thumb and thenar eminence and diagnosed to have atrial septal defect.

CONCLUSIONS : We successfully managed this case intra operatively for caesarian section under Epidural Anaesthesia.