The diagnosis of neuroendocrine tumors of the small intestine is usually challenging. They are infrequent, and the clinical course is insidious with nonspecific manifestations. Routine endoscopic and abdominal imaging studies are more often unremarkable. Therefore, distant metastases are frequently detected at the time of diagnosis. The tumor markers chromogranin A, synaptophysin, and neuron-specific enolase, and capsule endoscopy, and device-assisted enteroscopy are useful resources to establish a diagnosis. The aim was to present a case of neuroendocrine tumor of small intestine diagnosed with base in findings of the capsule endoscopy and further open surgery.