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Thrombotic thrombocytopenic purpura (TTP) was a mystery for over half a century until the discovery of ADAMTS13. ADAMTS13 is primarily synthesized in the liver, and it is a plasma protease responsible for the cleavage of von Willebrand factor (VWF), preventing the accumulation of ultra-large VWF (ULVWF) multimers that can spontaneously interact with platelets, causing microvascular thrombosis leading to thrombocytopenia and microangiopathic hemolytic anaemia. Deficiency of plasma ADAMTS13 activity (<10%) resulting from mutations of the ADAMTS13 gene or autoantibodies against ADAMTS13 causes hereditary or acquired (idiopathic) TTP. Pregnancy may be a precipitating factor in acute TTP, and there is a risk of relapse during subsequent pregnancies. TTP should be excluded in pregnancy-associated thrombotic microangiopathies. This is a case series of two siblings with known case of acute gravid TTP with genetic predisposition and suspected acquired component. Acute TTP is a medical emergency and requires a multidisciplinary team approach, especially when occurring during pregnancy or in the postpartum period. Clinical judgment and ADAMTS13 measurement are crucial for a correct diagnosis and an appropriate therapeutic approach.