Background:Pemphigus vulgaris is an autoimmune blistering disease that may initially present as localized lesions. It rarely remains localized throughout its clinical course.Observations: A 53-year-old woman with non-progressive pemphigus vulgaris localized to the tongue for 18 years is presented. Clinical examination showed erosions and ulcerations limited to the lateral margins of the tongue. Patient was treated with sublesional triamcinolone-acetonide injections as lesions recurred. Finally, triamcinolone- acetonide injections at three weeks intervals for three months induced a longterm sustained clinical remission for 18 months. The indirect immunofluorescence did not correlate with disease activity. Anti-desmoglein 3 antibodies (ELISA) remained elevated throughout the clinical course and during remission.Conclusions:This case highlights the recognition of localized pemphigus vulgaris and demonstrates the importance of local therapy and its potential to induce longterm remission. Similar report of additional cases may create a standard of care for non-progressive, localized pemphigus.