Kikuchi disease is a rare disorder with an unknown pathogenesis and a typically self-limiting natural course in predominantly previously healthy young women. Here we present a 54-year-old woman suffering from an overwhelming presentation of Kikuchi disease, associated with haemophagocytic syndrome, liver cell necrosis and nephrotic syndrome. She recovered fully without immunosuppressive treatment. This case report adds to the already broad clinical spectrum of Kikuchi disease described in literature. Awareness among physicians of the full clinical spectrum of Kikuchi disease and the self-limiting nature of this syndrome leads to a good diagnostic approach and may prevent initiation of longstanding immunosuppressive therapy.