Kikuchi Fujimoto disease (KFD) is a rare form of lymphadeopathy with systemic symptoms. We present a case of a 31-year-old female farmer who was admitted to the emergency ward because of swelling of the left arm. Upper extremity venous thrombosis due to local compression of lymph nodes was diagnosed. The histological workup of lymph node biopsy showed histiocytic necrotizing lymphadenitis which is a typical sign of KFD. Anticoagulant treatment for the venous thrombosis was initiated. As KFD is often associated with a transition to systemic lupus erythematosus follow up visits are scheduled in our outpatient clinic.