BackgroundKikuchi-Fujimoto disease (KFD) -also known as necrotizing histiocytic lymphadenitis -is a benign histiocytic lymphadenitis known for its low incidence and misdiagnosis that occurs mostly in young Asian females more than males. This disease resolves spontaneously in a few months with a low risk of relapse (one in 30 patients) after resolution. ObjectivesThe aim of this study is to share King Abdulaziz Medical City's (KAMC's) experience with KFD by determining its clinicopathological characteristics. Materials and methodsIn this study, we reviewed histopathological slides and pathological reports of all lymphadenopathy cases (683 cases) in the period between January 2008 and December 2018. ResultsForty-four cases of KFD were found and their clinicopathological characteristics were recorded. There is a slight female predominance (59% females versus 41% males) with a wide age range from 10 months to 97 years (mean = 28.8). The majority of the cases (63.6%) are seen in young adults (between 21 and 40 years). Association with autoimmune diseases was shown by 20.5% of cases while viral infection association was shown by few cases. Most cases showed remission (59%) and no deaths were reported upon follow-up. Histopathologically, the majority of cases have proliferative type followed by the necrotic type and few cases showed xanthomatous type. ConclusionsOur study has the largest number of KFD cases in this region. It is obvious that KFD has clinical, radiological, and pathological features that overlap with malignancy, especially lymphoma. Knowing this disease and careful diagnostic approach can help avoid misdiagnosis.