A young woman with progressive systemic sclerosis (PSS) and renal failure who received a renal transplant from her mother suffered accelerated loss of allograft function in the absence of hyperacute rejection or severe hypertension. A biopsy specimen and pathologic examination of the transplanted organ showed a fluorescent antibody pattern and vascular changes that were indistinguishable from those in the patient's native kidneys. This clinical sequence is a departure from the relative success of renal transplantation in the few previously reported cases of PSS where it has been used as therapy for renal failure.