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Objective We compared the efficacy and clinical outcomes of vindesine and prednisone (VP) and cyclophosphamide, etoposide, vindesine, and prednisone (CEVP) regimens as first-line treatment for multisystem (MS) or multifocal single system (SS-m) adult Langerhans cell histiocytosis (LCH). Method Clinical features, treatment response, and survival of adults with Langerhans cell histiocytosis treated at our center from January 2001 to January 2015 were reviewed retrospectively. Results Forty-five adult MS or SS-m LCH patients were treated ( N = 31, CEVP group; N = 14, VP group). Both treatment groups had similar gender distributions, patient ages, and extent of disease. The non-active disease rate for both groups was 70.0% and 64.3% ( P = 0.775), respectively. Median follow-up was 74.9 (range: 2.8–183.6) months and recurrence rates were 71.0% and 78.6% ( P = 0.593), respectively. The need for second-line therapy was 64.5% and 71.4% ( P = 0.649), respectively, and mortality rates were 9.7% and 15.4% ( P = 0.586), respectively. Neutropenia occurred in 48.4% of CEVP-treated patients and 7.1% of VP-treated patients ( P = 0.008). Conclusions CEVP or VP regimens for the treatment of adult SS-m or MS LCH showed similar efficacies, and both regimens were associated with high disease recurrence and the need for second-line therapy. [ABSTRACT FROM AUTHOR]