Abstract: Pancreatic cancer (PC) is a malignant digestive tract tumor with poor prognosis. Most of patients with PC are insensitive to traditional strategies of chemotherapy, targeted therapy and immunotherapy. PC with microsatellite instability‑high/deficient mismatch repair (MSI‑H/dMMR) is rare in clinic, which has distinctive clinicopathological characteristics and better prognosis from conventional PC. Reasonable acquisition of pancreatic tumor biopsy and accurate assessment of MSI‑H/dMMR status are helpful for accurate diagnosis of such patients. Individualized treatment strategy based on immunotherapy can significantly improve the prognosis of patients with MSI‑H/dMMR PC. Based on relevant literatures of domestic and foreign, the authors discuss the current status and research hotspots of diagnosis and treatment for MSI‑H/dMMR PC.