Kikuchi-Fujimoto Disease (KFD) is a self-limiting necrotizing lymphadenitis that usually presents with fever and cervical lymphadenopathy. Recognition of this condition is crucial, because it can be mistaken for tuberculosis, lymphoma and connective tissue disorders. When present at an unusual location the diagnosis can be challenging. We present an unusual case of Kikuchi-Fujimoto disease involving mesenteric lymph node masquerading as acute appendicitis along with its differential diagnosis. A 30-year-old female presented with complaints of acute abdominal pain, vomiting and fever. Physical examination revealed rebound tenderness in the right iliac fossa. The abdominal sonography was suspicious of acute appendicitis. The patient underwent appendectomy with excision of an enlarged mesenteric lymph node. On histopathology mesenteric node showed features of KFD which was confirmed on immunohistochemistry. Appendix was unremarkable. Although rare KFD should be added to the differential diagnosis of acute appendicitis in patients with enlarged mesenteric lymph nodes, Awareness of this disorder helps to prevent misdiagnosis and inappropriate treatment.