A newborn presented with haemolytic anemia, thrombocytopenia, hyperbilirubinemia and renal failure as early as the first hours of life. An early plasmatherapy was undertaken, followed by good outcome. The specific von Willebrand factor-cleaving protease (ADAMTS 13) was found at less than 5%. This is the specific biologic diagnostic element of congenital thrombotic thrombocytopenic purpura or Upshaw-Schulman syndrome. This disease of constitutional thrombotic microangiopathy was well identified and understood only few years ago. It's a rare disease which early diagnosis and treatment are crucial in order to preserve functional and vital capacities of the patient.