Objective To approach the recent advances in diagnosis and surgical treatment of pancreatic endocrine tumors （PETs）. Methods Articles relevant to diagnosis and treatment of PETs were collected and reviewed. Results PETs are characterized by their ability to over-produce peptides and hormones， which cause specific clinical syndromes. Because of rare incidence and complex clinical syndromes， there are still impediments to early diagnosis of these tumors. Monitoring of serum hormones and imaging method allow early tumor detection. PETs have been investigated for the past several decades. With the great knowledge of these tumors in molecular genetic level， clinical managements have been greatly changed. Conclusions Avoiding misdiagnosis is important for treatment of PETs. Surgical approach is still considered as the preferred option for curtailing the malignant progression of PETs and controlling the associated biochemical syndromes.