Kimura's disease is a chronic inflammatory pathology belonging to the angio-lymphatic proliferative disorders. The clinical features are a tumour-like swelling usually in the head and neck, with or without lymphadenopathy, often accompanied by eosinophilia and elevated serum IgE. It could be confused with angiolymphoid hyperplasia with eosinophilia. Other clinical differential diagnosis to Kimura's disease are reactive lymphadenopathy, lymphoma, parotid tumor with nodal metastasis and Mikulicz disease. The diagnosis is achieved by excisional biopsy which is also the therapy of choice. The prognosis is excellent. A case of Kimura's disease in an Italian female is presented together with a review of the literature.