Background Kikuchi-Fujimoto disease (KFD) is a benign, self-limiting disorder characterized by regional lymphadenopathy. Clinical symptoms range from mild fever and tenderness to upper respiratory syndrome. A few cases have been observed during pregnancy or Hashimoto's disease. What we describe here is the first observed case of KFD in a pregnant woman with a history of Hashimoto's thyroiditis. Case presentation A 36-year-old woman presented to Aleppo University Hospital during the 13th week of gestation with a painful cervical node on the right side of her neck. The patient's previous medical history confirmed Hashimoto's thyroiditis for several years. After histopathological examinations and radiological investigations, she was diagnosed with Kikuchi-Fujimoto disease and treated with corticosteroids. Although the patient did not adhere to the treatment very well due to her concerns for the fetus, the clinical picture improved after delivery. The patient now is on follow-up and continuing the current treatment with corticosteroids. Conclusions Further investigations need to be conducted to understand the possible autoimmune etiology of KFD when it is associated with Hashimoto's thyroiditis disease. It is also necessary to understand the relationship between this disease and pregnancy.