Introduction:
Kikuchi-Fujimoto disease (KFD), also called histiocytic necrotizing lymphadenitis, is a rare disease that occurs with swollen lymph nodes and associated fever. This disease occurs in both children and adults.

Aim:
The aim of our work was to review the literature and to remind family doctors, otolaryngologists, hematologists and rheumatologists about this rare disease that should be included in the differential diagnosis of long-term cervical lymphadenopathy.

Material and methods:
Current information on Kikuchi-Fujimoto disease was sought and presented based on literature that was published in reputable magazines in the period 2007–2020 in English. We searched for articles in the Medline, PubMed, and Scopus databases.

Results and discussion:
KFD occurs in both children and adults. This disease is found all over the world, most often in the Asian population. The etiology of Kikuchi-Fujimoto disease is not entirely known, however, two causal theories are suspected, which are discussed in detail in our article. The course of the disease is mild and usually disappears on its own. A biopsy of an involved lymph node presented as the standard for diagnosis. KFD treatment was causal – nonsteroidal anti-inflammatory drugs and/or glucocorticosteroids were used.

Conclusions:
There are few reports in the literature about Kikuchi-Fujimoto disease. KFD is associated with cervical lymphadenopathy and associated fever. If the diagnosis of the above-mentioned symptoms is not obvious, then in the differential diagnosis rarer diseases, such as KFD, should be included.