Neuroendocrine tumors (NETs) are rare tumors of the endocrine pancreas that require a high degree of suspicion for timely diagnosis. Diagnosis is often delayed due to the nonspecific and intermittent presentation of symptoms. As many as 45% to 55% of tumors are nonfunctional and are typically diagnosed secondary to mass effect related symptoms or found incidentally. Functional tumors often are symptom specific and are diagnosed at an earlier stage than nonfunctional tumors. The challenging aspects of treating NETs are localizing the tumors, treating extensive or metastatic disease, and palliating symptoms. Most NETs have an indolent course, and aggressive multimodality treatment is often indicated and encouraged.