Kimura's disease, which occurs endemically in the Far East and sporadically in the West, has so far eluded efforts to determine its exact pathogenesis. It presents as solitary or multiple benign swellings of the skin, has a predilection for the periauricular and scalp regions, and often is associated with regional lymphadenopathy. Morphologically, the lesions are characterized by proliferating blood vessels with rich eosinophilic infiltrate. Peripheral blood eosinophilia and raised serum IgE levels are signature features of the condition. The overall prognosis is good. When surgery is not possible, conservative treatment with either corticosteroids or radiation often can produce a favorable response. Complete surgical excision whenever feasible is the preferred treatment despite a high recurrence rate. Based on a recent case of Kimura's disease in a 55-year-old black woman, we discuss the pitfalls in the diagnosis of this chronic inflammatory disorder.