A 64-year-old Japanese woman developed therapy-resistant erythematous swelling of her upper lip. Our tentative clinical diagnosis of cheilitis granulomatosa was ruled out later by the laboratory findings including increased levels of anti-nuclear-antibody (ANA), anti-SSA/Ro antibody, and positive Schirmer test as well as by a histopathological picture showing a dense perivascular infiltration of plasma cells and mononuclear cells in the dermis instead of granulomatous changes. To the best of our knowledge, this is the first patient in whom annular erythema associated with S ogren's syndrome involved only the upper lip and produced clinical features simulating cheilitis granulomatosa.