Langerhans cell sarcoma (LCS) is an extremely rare malignant disease with multi-organ involvement and a poor prognosis. LCS involvement of tonsil has not been previously reported. However, we present herein a 10-year-old Tibetan boy distressed by LCS arising on the pharyngeal tonsil. Owing to local traditions and customs, he endured a long period of treatment with traditional Tibetan medicine which provided only slight relief at best that delayed an accurate diagnosis and scientific medical treatment. Subsequently, based on CT and MRI scans, combined with pathology features positive immunophenotype for the CD1a and S-100 proteins confirmed the diagnosed of LCS. We performed a surgical resection along with a regimen of E-CHOP chemotherapy was prescribed as new protocols. As a result the patient complete remission symptoms and without relapse has been 20 months.