Miescher's cheilitis granulomatosa is the monosymptomatic form of Melkersson-Rosenthal Syndrome. Severe macrocheilitis often causes a functional and esthetical impairment of the lip. Conservative treatment represents the first option to face this rare disease. Unfortunately, medical treatment is often ineffective without any significant result as far as swelling and disfigurement are concerned. Reductive cheiloplasty is indicated in all those patients who have failed to respond to medical treatment. Excision of excess tissue may be obtained by means of several surgical techniques. We report a case of a 55-year-old man affected by severe granulomatous cheilitis refractory to any medical treatment and then treated with the use of tongue flap for the reconstruction of the excised lower lip.