A patient with Fabry's disease underwent cadaveric renal transplantation. Unusual features of this case were as follows: (1) partial correction of the enzyme deficiency typical of the disease by the renal allograft; (2) the development of rapidly progressive pulmonary vascular obstruction and eventual fatal pulmonary embolism from subclavian vein thrombosis; and (3) surgical clipping of the inferior vena cava. This was done because of failure of and complications from anticoagulant therapy and did not in any way alter allograft function.