Posterior reversible encephalopathy syndrome (PRES) is defined by a spectrum of clinical symptoms and characteristic radiologic findings. Most patients show clinical recovery and normalization of imaging, even though a subset of rare cases may progress to cytotoxic edema, irreversible damage, and persistent radiological findings. As the condition is transient and seldom leads to death, few reports of pathologic findings during an acute episode or in patients following a resolved episode are available. Here, we describe the neuropathologic findings at autopsy in a 62-year-old woman with a history of systemic lupus erythematosus (SLE) and an episode of PRES that had resolved radiologically and clinically ~ 4.5 years prior to death. Autopsy findings included diffuse rarefaction of white matter with scattered microinfarcts, subpial gliosis, as well as perivascular lymphocytic aggregates and hemosiderin deposition in the sections from the posterior cerebrum. Some but not all of these changes of chronic white matter damage may be attributed to the coexisting systemic lupus erythematosus. Thus, we conclude that pathologic evidence of partial irreversible damage can be documented in PRES in spite of radiographic resolution of abnormalities.