To describe the clinical case of a 38-year-old man with BRAF negative and PD-L1 positive metastatic ATC, treated with pembrolizumab….After 3 months from the start of pembrolizumab and after 14 weeks from the start of lenvatinib, a reduction in thyroglobulin was detected (4906 ng/mL) and the results of vertebral MR and mandibular CT showed a reduction in metastases....Pembrolizumab combined with lenvatinib seemed to be effective in treating metastatic ATC and could become a therapeutic choice for patients presenting with PD-L1 expression.