Kikuchi-Fujimoto disease (KFD) is a rare and benign condition of unknown etiology affecting the lymph nodes. It primarily affects young adults aged 20 to 35 years, with a female predominance and a higher prevalence among individuals of Asian descent. It typically presents as painful lymphadenopathy, most often in the cervical region, and is associated with systemic symptoms such as fever, marked fatigue, and night sweats. Diagnosis relies on an excisional lymph node biopsy demonstrating necrotizing histiocytic lymphadenitis. KFD may be associated with underlying autoimmune diseases or reveal them, particularly systemic lupus erythematosus. Its clinical course is generally self-limiting, and management is mainly symptomatic.