Duodenal cancer is a clinically rare gastrointestinal malignant tumor. Its early symptoms lack specificity, making it difficult to diagnose clinically and easy to be misdiagnosed and missed. The treatment of duodenal cancer is mainly surgery, supplemented by radiotherapy and chemotherapy. Most patients have already experienced local invasion or distant metastasis at the time of first diagnosis, and lack of effective treatment, the prognosis is extremely poor. In recent years, a series of studies have pointed out that targeted therapy and immunotherapy can improve the prognosis of patients with duodenal cancer. This article reviews the progress of diagnosis and treatment of duodenal cancer, hoping to help guide clinical diagnosis and treatment and develop new treatment options.