Hemophagocytic lymphohistiocytosis (HLH) is an uncommon life-threatening condition caused by an uncontrolled immunological response. It can develop secondary to malignancies, infections, systemic diseases, and immunosuppression. Multiple risk factors may present in kidney transplant recipients; however, the cases of HLH in this population have been described sparsely. We report a case of a 39-year-old female kidney transplant recipient who presented to the hospital nearly 3.5 years after the transplantation with general malaise, recent history of weight loss, fevers, and persistent anemia. Laboratory tests showed pancytopenia, hyperferritinemia, hypertriglyceridemia, and increased activity of lactate dehydrogenase. A bone marrow aspiration revealed hemophagocytosis, which led to the diagnosis of HLH. Therapy consisting of high-dose steroids and plasma exchanges was administered, resulting in a significant improvement of blood count parameters and the patient’s general condition. While searching for the triggering disease, a single cavitary lesion in the right lung was revealed in a chest radiograph. Computed tomography scan, bronchoscopy, and additional laboratory testing did not reveal a definitive cause of the lesion. We suspect that the lesion may be a consequence of HLH. The patient was disqualified from thoracic surgery due to multiple comorbidities. Even though HLH is a rare condition, it should be taken into consideration in a kidney transplant patient presenting with unspecific symptoms accompanied by a bicytopenia. It has an unpredictable course that often results in serious complications. Thus close follow-up of the patient and a wide array of imaging and laboratory tests remain crucial.