Treatment of Langerhans cell histiocytosis (LCH) is yet to be established. We treated seven patients with etoposide alone at a dose of 100 mg/m2/day for 3 days given every 3 to 4 weeks for six cycles. Three patients had received prior chemotherapy, two patients were less than 2 years of age, and two had liver dysfunction. A positive response to therapy was seen in five patients. There was no major toxicity. Etoposide therapy is safe and effective in the treatment of LCH.