Gastroenteropancreatic neuroendocrine tumors (GEP-NET) are a heterogeneous group of neoplasms that arise from neuroendocrine cells of the GI tract and pancreas. Due to the lack of symptoms in the early stage of the disease and the frequency of nonspecific gastrointestinal symptoms, GEP-NET are difficult to diagnose. This delay in diagnosis often results in patients presenting with advanced disease and thus a poor prognosis. There is an unmet medical need for earlier, more definitive GEP-NET diagnosis. Identification of effective biomarkers to improve GEP-NET diagnosis, as well as to assess treatment efficacy, relapse and prognosis, is important for improving outcomes in GEP-NET. Chromogranin A is currently the most useful general biomarker for the assessment of GEP-NET. This review summarizes the biochemical characteristics of chromogranin A, its specificity and sensitivity for GEP-NET diagnosis, and its use in monitoring treatment effectiveness, disease progression and prognosis.