Kikuchi-Fujimoto disease is characterized by painful cervical lymphadenopathy and constitutional symptoms. Microscopical study of lymph nodes shows focal areas of non-suppurative necrosis with histiocytic and plasmacytoid cell infiltrates. The course is usually benign. Often primitive, necrotising histiocytic lymphadenopathy may be associated with autoimmune disorders. We describe the case of a 30-year-old female patient with two 15-day courses of Kikuchi-Fujimoto disease flares within a period of 3 months, occurring in association with mixed connective tissue disease.