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Intrapancreatic accessory spleen: an enigmatic entity. Solitary hypervascular lesion in the distal body/tail of pancreas in a patient with non-specific abdominal symptoms is a diagnostic challenge. Neuroendocrine neoplasm (NEN) and metastasis from renal cell carcinoma are the most common differentials and intrapancreatic accessory spleen (IPAS) is the rarest of its differential diagnosis. We present, here, a case of a 56-year-old man with a space-occupying lesion in body/tail of pancreas that was preoperatively diagnosed as a NEN based on elevated chromogranin levels and hyperenhancing lesion on contrast-enhanced CT scan. He underwent a spleen-preserving distal pancreatectomy. The final histopathology revealed an IPAS.