© 2021 Acta Gastroenterológica latinoamericana.Thrombotic Thrombocytopenic Purpura is a syndrome of microangiopathic hemolytic anemia accompanied by thrombocytopenia, neurological disorders, renal failure and fever. Acute pancreatitis is a rare cause of Thrombot-ic Thrombocytopenic Purpura and this manifestation, at the same time, is a rare complication of acute pancreati-tis. Thrombotic Thrombocytopenic Purpura is induced in acute pancreatitis by poorly understood mechanism, which involves multiple pathways apart from only ADAMTS-13 deficiency. Here, we analyze the case of a 47-year-old female who presented with an acute pancreatitis. She was diagnosed with Thrombotic Thrombocytopenic Purpura and an acute pancreatitis at the same time, with throm-bocytopenia and peripheral smear findings at presentation. Therefore, Thrombotic Thrombocytopenic Purpura secondary to the pancreatitis was considered in this case. In this work, we have discussed details of our case and the dif-ferent mechanisms involved in pathogenesis of Thrombotic Thrombocytopenic Purpura in acute pancreatitis and their outcome with prompt management.