Neuroendocrine neoplasms (NENs) of the pancreas are rare and frequently malignant. Our presentation of a pancreatic NEN analyzes the diagnosis circumstances, staging, treatment, one-year evolution and disease particularities. A 39-year-old nonsmoker patient was admitted in the Clinic of Pulmonology, Tirgu Mures, Romania with a pneumonia suspicion (fever, thoracic pain irradiated below the diaphragm, mild dyspnea). The chest X-ray showed a rise of the left diaphragm. Abdominal ultrasound revealed a large pancreas-related tumor. Computerized tomography (CT) scan with contrast confirmed a well-vascularized pancreatic tumor, which invades spleen, collateral circulation of the splenic vein, enlarged liver without secondary lesions and no retroperitoneal adenopathies. The patient was referred to the surgery where there was performed total tumor resection, spleen resection, and large lymphadenectomy. Histopathology and immunohistochemistry revealed the pancreatic NEN G2 grade, T3N1M0 and allowed accurate treatment. 2010 World Health Organization (WHO) NENs classification recommends further treatment-related biomarkers determination only in selected cases. Our case evolution after one year was favorable without local tumor relapse or metastases. The close survey of the patient (by clinical exam, imaging and biological markers) is ongoing. The onset of asymptomatic pancreatic tumor may have atypical respiratory symptoms. Imaging methods (ultrasound, contrast CT) are recommended in borderline symptomatology. Radical surgical resection of the tumor with lymphadenectomy, histopathology with immuno-histochemistry play an essential role in the correct diagnostic, grading, staging and treatment of pancreatic NENs. Close survey of the clinical, imagistic and biological markers is recommended.