Congenital factor VII deficiency is a rare bleeding disorder, with an incidence of 1:500,000. This case report describes an exceptionally unusual combination of congenital factor VII deficiency, multicystic dysplastic kidney disease and bicuspid aortic valve disease, in the same patient.

Pseudothrombotic microangiopathy is thrombocytopenia, anemia and schistocytosis caused by Cobalamine deficiency. It could be easily misdiagnosed as microangiopathic hemolytic anemia. Evaluation of lactate dehydrogenase, reticulocyte count, peripheral blood smear, platelet count, cobalamine and homocysteine level are crucial for differentiating pseudothrombotic microangiopathy from a true microangiopathic hemolytic anemia. A case of pseudothrombotic microangiopathy due to severe vitamin B12 deficiency is presented.