Lily leaf spot is an emerging disease of the globally rare Lilium grayi S. Wats., a species endemic to the southern Appalachian Blue Ridge, USA. The species is considered Threatened or Endangered in the three states where it naturally occurs (North Carolina, Tennessee, Virginia). Disease leads to premature senescence of aboveground tissues and curtailment of sexual reproduction. Spore morphology, completion of Koch’s postulates, and DNA sequence data showed the causative agent of lily leaf spot to be Pseudocercosporella inconspicua (G. Winter) U. Braun, a Lilium-specific basidiomycete. Diagnostic disease symptomatology includes amphigenous necrotic lesions with tan to green margins encircling a white to grey powdery mass of conidia. Studies conducted in the field at Roan Mountain, NC/TN, in the largest known population, showed that a visual disease diagnosis based on morphology can be highly accurate in predicting P. inconspicua infection, and that high concentrations of conidia of P. inconspicua are strongly associated with infected L. grayi, but are largely absent or few on uninfected L. grayi and other species. Field inoculation trials using infected L. grayi leaf tissue as inocula resulted in transmission of disease and induction of premature senescence under natural conditions.