Patients diagnosed with advanced adenoid cystic carcinoma (ACC) with metastasis 
to the lung generally have poor prognosis when they exhibit resistance to 
conventional therapies. Programmed cell-death protein 1 (PD-1) inhibitors, a 
type of Immune checkpoint inhibitors (ICI), have shown good response in the 
treatment of various types of malignant tumors; however, objective response 
rates of monotherapy for advanced ACC are low. Anlotinib, a novel, orally 
managed tyrosine kinase inhibitor, that targets vascular endothelial growth 
factor receptor (VEGFR), fibroblast growth factor receptor (FGFR), 
platelet-derived growth factor receptor (PDGFR), and c-kit, has appeared great 
adequacy in treating numerous sorts of malignant tumors, particularly tumors 
with lung metastases. Here, we have presented a case of refractory ACC with lung 
metastases that was reduced after combinatorial treatment using the immune 
checkpoint inhibitor (ICI) toripalimab and anti-angiogenesis agent anlotinib. 
The patient achieved a reduction in lung metastases by chest computed tomography 
(CT) examination, with an outcome of stable disease (SD) of 5 months, a 
significant decrease in the levels of peripheral blood cytokines interleukin 6 
(IL-6) and tumor necrosis factor-α (TNF-α), as well as good tolerance without 
noteworthy unfavorable reactions, indicating that the combined therapy of 
toripalimab and anlotinib may be utilized in the management of advanced ACC.