Vitamin B12 deficiency may present as pseudothrombotic microangiopathy. Pseudothrombotic microangiopathy is anemia, thrombocytopenia, and schistocytosis caused by vitamin B12 deficiency. Pseudothrombotic microangiopathy may be mistaken for microangiopathic hemolytic anemia, confounding appropriate treatment. Evaluation of lactate dehydrogenase, reticulocyte count, bilirubin, and platelet count are most helpful in differentiating pseudothrombotic microangiopathy from a true microangiopathic hemolytic anemia. A case of pseudothrombotic microangiopathy due to severe vitamin B12 deficiency is presented.