Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy caused by severely reduced activity of the von Willebrand factor-cleaving protease ADAMTS13. Over 95% are acquired autoimmune TTP. In children, acquired TTP is a very rare, life-threatening disease. To date, no consensus exists on the treatment strategy of pediatric TTP. In our experience, the combined use of PEX, Caplacizumab and immunosuppressive therapy during the acute phase of the disease results in a prompt and persistent normalization of laboratory findings and successful clinical outcome.