Tehran University of Medical Sciences Case Reports in Clinical Practice 2538-2683 9 6 2025 07 01 277 280 Asrith Reddy Gandu Department of Medicine, Ramaiah Hospital, Hyderabad, Telangana, India. Amarender Reddy Nagala Department of Medicine, Ramaiah Hospital, Hyderabad, Telangana, India. Pavan Reddy Guggila Department of Medicine, Ramaiah Hospital, Hyderabad, Telangana, India. 2025 03 21 2025 04 07 Kikuchi-Fujimoto disease (KFD) is a rare, self-limiting condition characterized by fever and lymphadenopathy. Its association with Sjögren’s syndrome (SS) is exceedingly rare, particularly in the absence of classical sicca symptoms. This case report describes a young male with KFD and SS, highlighting the diagnostic challenges and the importance of a multidisciplinary approach in atypical presentations. A 21-year-old male presented with a 20–25-day history of intermittent high-grade fever, chills, generalized weakness, and severe epigastric pain. Initial investigations suggested enteric fever, but the recurrent symptoms prompted further evaluation. Imaging revealed widespread lymphadenopathy, and histopathological examination confirmed the diagnosis of KFD. Serologic testing revealed SS despite the absence of dry eyes or mouth. The patient was managed symptomatically, and his condition improved with supportive care. This case highlights the importance of considering rare autoimmune etiologies in young patients with persistent febrile illnesses and atypical systemic features. The concurrent diagnosis of KFD and SS without classical sicca symptoms adds to the limited literature on this rare association. A multidisciplinary approach, including histopathological and serological testing, is crucial for accurate diagnosis and management. Further research is needed to better understand the pathogenesis and autoimmune associations of KFD. https://crcp.tums.ac.ir/index.php/crcp/article/view/1034 https://crcp.tums.ac.ir/index.php/crcp/article/download/1034/700