Back ground: Holt–Oram is rare autosomal disorder, clinically characterised by congenital cardiac defects and morphological abnormalities of the upper limbs.

Case characteristics:10 days old neonate born to first degree consangunious parents presented with upper limb anomaly and cardiac defects.

Obseravations : Hypoplastic left thumb with ASD, VSD and PDA was present.

Message– Neonate with upper limb anomaly should be evaluated for congenital hear disease.