A total of five cases from three different families with selective vitamin B12 malabsorption and proteinuria are presented. Two cases were brought to the hospital because of anemia and heart failure and one for evaluation of the anemia. Two additional cases were detected during family studies among the siblings of two different cases. All three index cases had abnormal Schilling test, none of which were corrected by IF. The anemias responded to parenteral vitamin B12 therapy. In the affected siblings, megaloblastic anemia was present; a Schilling test was not performed. All five cases and three of the parents had proteinuria without any obvious kidney abnormality. One of the cases also had tapeto-retinal dystrophy.