Cystic tumors of the pancreas are relatively rare, and are often misdiagnosed or neglected in clinical practice due to slow growth, deep location and the lack of typical symptoms and signs. With the development of pancreatic surgery and imaging techniques in recent years, as well as the continued investigation of pancreatic cystic tumors, considerable progress has been achieved in this field. These have promoted the early detection, treatment planning, and choice of surgical procedure and estimating the prognosis for pancreatic cystic tumors. This paper reviews the advances in clinical diagnosis and treatment of these diseases.