Melkersson-Rosenthal Syndrome (MRS) is a systemic neuro-mucocutaneous 
granulomatous disease, characterized in its classical form by a triad of 
recurrent facial nerve paralysis, swelling of the lips and lingua plicata. 
However, this classical triad is rarely present, while the monosymptomatic or 
oligosymptomatic forms are more frequent. The presence of two or one of the 
manifestations mentioned above, with granulomatous cheilitis in the biopsy, is 
sufficient to make the diagnosis of monosymptomatic or oligosymptomatic form of 
MRS. This syndrome is very rare in childhood, instead, it is more frequent in 
young adults between the second and third decades of life. We present the case 
of an 8 years old boy who was brought to us because of a non painful swelling of 
the upper lip, associated with gingival hypertrophy, that had persisted for more 
than two months. Given the negative results of the hemato-chemical and 
instrumental assessments, we performed an upper lip biopsy whose histological 
study showed granulomatous cheilitis. We diagnosed this case as a 
monosymptomatic MRS and administered an intralesional steroid therapy using 
triamcinolone, with complete recovery.