Kikuchi's disease is an idiopathic self-limiting condition first reported in Japan in 1972. However, hemophagocytic lympho-histiocytosis is a condition that occurs due to overstimulation of the immune system. The presence of the two conditions is rare, and the clinical observation of this unusual clinical syndrome is worth reporting. We are reporting an 18-year-old Saudi female patient who presented with high-grade fever and diaphoresis 3 weeks before her presentation. Physical examination showed palpable cervical and axillary lymphadenopathy; laboratory investigation found neutropenia, a high lactate dehydrogenase of 550 U/L, and high ferritin levels. A thoracoabdominal computed tomographic study revealed generalized lymphadenopathy. She was diagnosed with hemophagocytic lympho-histiocytosis based on a bone marrow biopsy finding and Kikuchi's disease based on an excisional cervical lymph node biopsy. She received a high dose of dexamethasone with complete resolution of the condition. In conclusion, hemophagocytic lympho-histiocytosis and Kikuchi's Disease are uncommon conditions. The presence of a combination of such two conditions is extremely rare and worth reporting. Early diagnosis and initiation of the management with high dexamethasone dose could save patient life.