Background: Kikuchi-Fujimoto Disease (KFD) is a rare disease and is commonly seen in Asian population. It usually presents with prolonged fever, leukopenia and persistent cervical lymphadenopathy. It is a benign disease with a female preponderance. The cause is unknown but many theories have been postulated, like autoimmune, inflammatory, infectious agents and molecular mimicry. Most of the patients will recover themselves without any sequelae, in six-month time. However, it may rarely also convert to autoimmune disease, like SLE. The recurrence rate is very low. It is important to consider it as a differential diagnosis in chronic persistent lymphadenopathy, like tuberculosis, lymphoma, HIV and autoimmune lymphadenitis. Cases: Here we present a case of a 37-year-old female who initially went to a private practitioner with chief complaint of a persistent neck mass of approximately 6 months duration, multiple small joints pain and persistent fever. She underwent excisional biopsy for suspected lymphoma, but final pathology rendered a diagnosis of KFD. Second case also presented with multiple neck swelling and joint pains, suspect of SLE, connective tissue disorder or young-onset still's disease was made clinically but excisional biopsy revealed KFD. Both the patients improved with NSAIDS and low dose wysolone therapy. Conclusion: The purpose of this article is not only to review the literature but also to create awareness of this entity in the differential diagnosis of persistent lymphadenopathy, especially for the general otolaryngologist in a community-based setting. In addition, this review would be beneficial for other practitioners as well, specifically paediatricians, infectious disease physicians, rheumatologists, pathologists, and medical oncologists.