Lymphangiomas of the tongue are rare, and their treatment is problematic. A 10 year-old patient with tongue lymphangioma who was previously treated with surgery and propranolol with no response was treated with sirolimus in our department. We used sirolimus with a dose of 1.6mg/m(2)/day. After 3 months of treatment, the mass had decreased by more than 60%. We continued the treatment for 1 year with a maximum response of 70% decrease in mass. Disease remained stable 6 months after stopping therapy, the latest time of follow-up. Sirolimus appears to be effective in lymphangioma but requires further study. Pediatr Blood Cancer 2014;61:931-932. (c) 2013 Wiley Periodicals, Inc.