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Background: Melkersson-Rosenthal syndrome is a rare disorder that is characterized, in its full form, by recurrent facial nerve palsy, fissured tongue, and orofacial edema. Most cases present as oligosymptomatic or monosymptomatic forms. Its etiology is still unknown and its course is chronic and it may be progressive., Case Report: We present the case of a nine-year-old girl with recurrent episodes of peripheral facial nerve palsy. During the study, lip edema, benign migratory glossitis, and angular cheilitis were observed, which is why a clinical diagnosis of Melkersson-Rosenthal syndrome was made., Conclusions: This syndrome must be considered in the differential diagnosis with the presence of acute peripheral facial nerve palsy and/or facial edema due to its behavior and progressive evolution.