1.1 Background: The vast majority of the reported cases of congenital absent radii without thumb aplasia were associated with congenital thrombocytopenia or thrombocytopenia occurring early life. The occurrence of congenital absent radii without thumb aplasia and without evidence congenital thrombocytopenia or thrombocytopenia occurring early life is extremely rare and has not been reported in Iraq. 

1.2 Patients and methods: An Iraqi newborn infant with congenital absent radii without evidence of congenital thrombocytopenia during the first week is presented, and the relevant literatures were reviewed with aim of describing the early documentation of the condition in the medical literature. 

1.3 Results: A newborn infant with congenital absence of the radii without thumb aplasia. The neonate was followed for one week without the occurrence of thrombocytopenia. The rest of physical examination was normal and no other abnormalities could be detected. The parents were consanguineous, but family history was negative for a similar condition. 

1.4 Conclusion: The extremely rare occurrence congenital absence of the radii without thumb aplasia without evidence of thrombocytopenia during the first week of life is reported in an Iraqi newborn.