Kikuchi-Fujimoto disease (KD), also known as histiocytic necrotizing lymphadenitis, is a rare cause of unilateral cervical lymphadenopathy usually described in adolescents and young adults with female preponderance. Clinically patients present with palpable lymphadenopathy, fever, and loss of weight. Hence, it should be differentiated from other causes of lymphadenopathy such as tuberculosis, lymphoma, or malignancy. Although the majority resolve spontaneously without treatment some may require non-steroidal anti-inflammatory drug or steroids. It may also be associated with systemic lupus erythematosus. Hence early detection, prompt diagnosis, and follow-up of the patient are essential in all cases of unilateral cervical lymphadenopathy in young adults. Here, we describe a 22-year old female who presented with unilateral neck swelling and was clinically diagnosed initially as a case of tuberculous lymphadenitis which on biopsy turned out to be KD.