Congenital Thrombotic Thrombocytoenic urura (TT) is a rare disease with diverse presentation that can also mimic Idiopathic Thrombocytoenic urura (IT) and be misdiagnosed. After confirming diagnosis with ADAMTS13, it can sipmly be treated with FF transfusion and complications can be prevented. We describe an eight year old girl previously managed as IT and referred to Children Cancer Hospital for opinion. History, clinical examination and laboratory findings were not consistent with IT. History of neonatal jaundice, microangioathic haemolytic anaemia and thrombocytoenia favoured congenital TT. Low ADAMTS13 level and improvement in platelet counts after FF transfusion confirmed the diagnosis of congenital TT. So cases with atypical presentation of IT should be properly investigated to make correct diagnosis and avoid unnecessary exposure to drugs and their complications.