Background <p>Pancreatic paraganglioma (P-PGL) is an exceptionally rare neuroendocrine tumor, often misdiagnosed as other pancreatic neoplasms due to overlapping clinical and radiological features. Evidence is limited to case reports and small series, and standardized diagnostic and therapeutic strategies are lacking.</p> Objective <p>To systematically review published cases of P-PGL, summarizing clinical presentation, diagnostic approaches, management strategies, and outcomes.</p> Methods <p>A systematic literature search was performed in major biomedical databases to identify reports of P-PGL with histological confirmation. Data regarding demographics, clinical and biochemical characteristics, imaging findings, treatment modalities, and follow-up were extracted and analyzed descriptively.</p> Results <p>A total of 50 cases were identified, predominantly in middle-aged adults, with a slight female predominance. Most tumors were non-functioning and incidentally discovered, although a subset presented with symptoms related to catecholamine excess. Imaging findings were heterogeneous and frequently indistinguishable from pancreatic neuroendocrine tumors. Surgical resection was the primary treatment in most cases and was associated with favorable outcomes. Malignant behavior was uncommon but reported. Long-term follow-up data were scarce.</p> Conclusions <p>P-PGL is a rare entity with nonspecific clinical and radiological features, posing significant diagnostic challenges. Definitive diagnosis relies on histopathology and immunohistochemistry. Surgical resection appears to be the treatment of choice, with a generally good prognosis. Greater awareness and systematic reporting are needed to improve understanding of its natural history and optimal management.</p>