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Background: Miescher's cheilitis, whether occurring alone or as part of Melkersson-Rosenthal syndrome, is a rare type of granulomatous inflammation of unknown cause with an even rarer genital equivalent. Herein, we describe a case of the latter condition developing over a 12-year period., Patients and Methods: A 27-year-old woman presented episodes of unilateral vulvar oedema, which initially regressed but resulted over time in permanent vulvar and perineal tumefaction. Histology revealed epithelioid histocytic granulomas so mild that they were not noticed at the start of the disease. The oedema remained the sole sign for 12years and two gastrointestinal screening tests for Crohn's disease proved negative, diagnosis of the latter condition was ruled out., Discussion: Miescher's granulomatous vulvitis requires differential diagnosis, essentially with regard to Crohn's disease. Our case illustrates the difficulty in diagnosing this rare disease as well as the uncertainties surrounding its physiopathology. Diagnosis rests upon repeated sampling and biopsies with repeated levels of sections in order to identify the characteristic perivascular granulomas, which may be very mild., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)