Congenital ADAMTS-13 deficiency is rare, with only between 150 and 200 living cases described internationally. It can present in the neonatal period with thrombocytopenia that may be associated with thrombosis rather than haemorrhage, microangiopathic haemolytic anaemia (MAHA) and jaundice requiring exchange transfusion. We report a case of a large cerebral infarction resulting from severe ADAMTS-13 deficiency in the immediate neonatal period. The diagnosis of ADAMTS-13 deficiency should be considered in babies with haemolytic anaemia, jaundice, thrombocytopenia and a negative direct antiglobulin (Coombs) test (DAT). It is important to diagnose and treat early in order to prevent further brain and kidney damage.
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