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Langerhans' cell histiocytosis (LCH) is a condition in which granulomas form in various tissues through the accumulation of abnormal histiocytes (Langerhans' cells), granulocytes and lymphocytes. An important distinction between LCH and primary pulmonary histiocytosis (PPH) is that smoking cessation is known to often lead to spontaneous improvement in PPH patients, while this has not been demonstrated in patients with multisystem LCH. In this case report, we describe the case of a 20-year-old man who presented with cough and palpably enlarged lymph nodes in his neck. An inguinal lymph node biopsy led to the diagnosis of multisystem LCH in the lungs, the cervical, intra-abdominal, and inguinal lymph nodes and the spleen. The patient was a smoker, and had averaged 30 cigarettes per day for 7 years. To address the multisystem involvement, intravenous pulse steroid therapy was administered (30–40 mg/kg daily) in two 3-day treatment periods separated by 3–4 weeks. The patient was also advised to stop smoking, and did so. Serial follow-up examinations confirmed an objective improvement with complete remission. The patient was still healthy at 18 months post-treatment, reflecting a dramatic and positive response to pulse steroid therapy. This encouraging result makes this case an important one to highlight.