Emine Uslu,1 Sibel Gurbuz,2 Abdulsamet Erden,1 Fatma Aykas,1 Hatice Karagoz,1 Samet Karahan,1 Hatice Karaman,3 Ali Cetinkaya,1 Deniz Avci1 1Internal Medicine Department, 2Infection Disease Department, 3Clinical Pathology Department, Kayseri Training and Research Hospital, Kayseri, Turkey Abstract: Kikuchi disease, also called Kikuchi–Fujimoto disease or Kikuchi's histiocytic necrotizing lymphadenitis, is a rare, benign condition of unknown cause, usually characterized by cervical lymphadenopathy and fever. The diagnosis is based on histopathology. Our patient was a woman with bilateral cervical lymphadenopathy, fever, chest and abdominal pain, fatigue, maculopapular rash on her face, trunk, and upper and lower extremities. Immunological and rheumatological tests were negative. We took a cervical lymph node biopsy that showed a proliferative and necrotizing process centered in the paracortex characterized by patchy circumscribed or confluent areas of necrosis associated with karyorrhexis, and was remarkable by the absence of granulocytes and the paucity of plasma cells. These findings confirmed the diagnosis of Kikuchi's disease. The patient's hemoglobin values decreased, and the peripheral blood smear revealed schistocytes. Blood tests showed raised D-dimer, activated partial thromboplastin time, prothrombin time, and international normalized ratio with decreased fibrinogen. The patient's condition quickly worsened and disseminated intravascular coagulopathy eventually developed. Her initial management consisted of a corticosteroid and hydroxychloroquine. Keywords: disseminated intravascular coagulopathy, Kikuchi–Fujimoto disease, necrotizing lymphadenitis