Successful treatment of infants with gastrointestinal involvement in Langerhans cell histiocytosis (LCH) has been poor, with no specific chemotherapeutic regimen of clear benefit. An 8-month-old male, diagnosed with LCH by skin and gastrointestinal biopsies, was treated with several cycles of 2-chlorodeoxyadenosine, vinblastine and prednisone with only partial response. Ultimately, two cycles of 2-chlorodeoxyadenosine concomitant with high-dose cytarabine led to a durable complete response. Twenty-seven months since the last course of chemotherapy, the patient continues to thrive free of disease. Treatment with 2-chlorodeoxyadenosine and cytarabine should be considered for further study in patients with poor-prognosis LCH.