Human microsporidiosis has been described most commonly in patients with acquired immunodeficiency syndrome and only rarely in those with other forms of immunosuppression. Only 11 cases of microsporidiosis have been reported previously in solid transplant recipients. To our knowledge, this is the first report to describe a case of microsporidiosis in a pancreas/kidney transplant recipient in whom multi-organ system dissemination was observed. This infection was not detected until postmortem examination of stained tissue sections revealed microsporidian spores that were identified as Encephalitozoon species by transmission electron microscopy. It is suspected that leakage from the duodenal anastomosis to the bladder may have contributed to the dissemination of this infection.