Melkersson–Rosenthal syndrome is traditionally defined as a triad of syndromes including recurrent facial nerve palsy, oedematous changes within face and a geographic tongue. Aetiology of the disease is not definitely confirmed. Probably it is multifactorial with marked influence of autoimmune response of delayed hypersensitivity type. It is also claimed that infections, systemic disorders as well as trauma may be relevant. The genetic predisposition cannot be excluded. Cases with full clinical presentation are relatively rare, the incomplete cases are problematic for establishing diagnosis and treatment despite the fact that case report of the syndrome are more numerous nowadays. The article presents a case of patient with a recurrent facial nerve palsy and scrotal tongue, in whom the diagnosis was established after 18 years after the first sign occurred. There was a differential diagnostics conduced and the final diagnosis of incomplete Melkersson–Rosenthal syndrome was established. Standard therapeutic strategy was introduced but satisfactory results were not obtained. This case report highlights the diversity of clinical manifestation of the syndrome effecting from a sequence of their appearance, the variability of therapeutic theories, lack of standardized guidelines for therapeutic strategies and importance of collaboration between doctors of different specialties to elucidate this rare diagnosis.