Kikuchi disease (histiocytic necrotizing lymphadenitis) is a rare, benign entity which predominantly affects young women. The disease usually presents with painful or painless enlarged cervical lymph nodes accompanied with fever. The cause of the disease is uncertain and only 17 cases in the literature have been reported in the past to be associated with systemic lupus erythematosus (SLE). We report four cases of SLE with Kikuchi disease in Taiwan. This is the first report regarding Kikuchi disease and SLE in Taiwan. All patients are female. The mean age is 30 (ranging from 21 to 35 years). The mean history of SLE is 4 years (ranging from 10 days to 8 years). Three of our patients (75%) developed Kikuchi disease accompanied with flare-up of lupus activity, and the other one had Kikuchi disease simultaneously with the onset of SLE. One patient had ribosomal-P antibodies, one had ribonuclear protein (RNP) antibodies and none had antibodies to Ro(SSA) or La (SSB). The cause of association of Kikuchi disease with SLE is still unknown. From our cases, the high frequency of flare-up of lupus disease activity accompanied with the onset of Kikuchi disease and the simultaneous occurrence of these two diseases indicate that they are not independent events. We speculate that Kikuchi disease may be one of the manifestation of SLE.