Dermatopathic lymphadenitis (DL) is considered a benign disease, the symptoms of which can be relieved with only anti-histamine. It can be confused with lymphoma. Here, we report a case of DL, which resulted in hemophagocytic lymphohistiocytosis (HLH). After adequate treatment, remission was obtained at first; however, recurrence with liver failure occurred six months later, which quickly resulted in death. This particular case has not been reported elsewhere. It reminds us that even benign dermatological problems can cause life-threatening syndromes, such as, in this case, HLH. The risk needs to be taken seriously in patients presenting with fever, skin problems, and lymphadenopathy.