BackgroundSarcomatoid carcinoma of the pancreas (SCP) is a rare type of malignant pancreatic neoplasm, and its prognosis is even worse than that of conventional pancreatic ductal adenocarcinoma (PDAC). Currently, there is no standard regimen for treating SCP, and the impact of systemic therapy on the survival of patients with SCP has not been well defined.Case presentationHerein, we report a 38-year-old Asian man diagnosed of local unresectable SCP with supraclavicular lymph node metastasis, radical excision after camrelizumab and anlotinib therapy, which resulted in a remarkable reduction in the size of primary tumor and complete remission of the metastatic lymph node.ConclusionsThis is the first report of the use of immunotherapy and anti-angiogenesis therapy in a patient with SCP, which provides optimistic data to support the synergistic effect.