BACKGROUND Difficulties in making an accurate preoperative diagnosis of cystic pancreatic lesions pose a challenge for radiologists. It would be helpful to report rare cases and review the literature. CASE SUMMARY In the present report, a case of a patient with a pancreatic cystic lesion initially misdiagnosed as a pseudocyst by radiologist was documented, which was later pathologically confirmed as pancreatic ductal adenocarcinoma with neuroendocrine tumor. However, subsequent literature review yielded no previous reports of pancreatic ductal adenocarcinoma with neuroendocrine tumors and cystic lesions. Therefore, literature on the imaging diagnosis of pancreatic cystic lesions was instead reviewed and discussed. CONCLUSION Careful evaluation of the characteristics revealed by multimodal imaging techniques, medical history, laboratory examination data and follow-up observations, is critical to the diagnosis and treatment of pancreatic cystic disease. We provide valuable insights into the diagnosis of pancreatic cystic disease through a rare case report and literature review.