1 Department of Pediatrics, Memon Medical Institute Hospital, Karachi –74800, Pakistan.
2 Department of Pediatrics, Bahria University Medical and Dental College, Karachi –74800, Pakistan.
3 Department of Pediatrics, Indus Hospital, Karachi –74800, Pakistan.
Hemophilia is one of the most common inherited coagulation disorder; with almost half of the cases presents in the neonatal period with bleeding episodes specially post circumcision, although family history is present in about two third cases. Retroperitoneal hemorrhage is a very rare presentation of hemophilia and to the best of our knowledge has not described yet, as initial presentation of hemophilia B in the neonatal period. We are presenting retroperitoneal hemorrhage as initial presentation of hemophilia B, in the presence of family history which has not been disclosed till the baby become symptomatic.