Kikuchi-Fujimoto disease (KFD) is a histocytic necrotizing lymphadenitis of cervical lymph node, which is usually combined with tenderness and fever presents mainly in women in their thirties. KFD has been rarely associated with SLE and the microscopic features of KFD can be similar to SLE. We described the case of a young man, originally diagnosed as having KFD by lymph node histology, who subsequently developed SLE with a skin rash, oral ulcer, hematologic and immunologic disorder and high titer of ANA. We recommend study of sexual and age-specific differences in clinical progress of KFD, and a long-term follow-up evaluation is also needed.