In this paper, I report a rare, low-grade malignant tumor, solid and papillary epithelial neoplasm of the pancreas (SPENP). I also discuss and review 157 previously reported cases. Unlike other malignant tumors of the pancreas, this neoplasm is typically found in young women, does not have metastases, and is amenable to cure after complete surgical resection. I discuss clinical features, diagnostic procedures, and differential diagnosis. Fine-needle aspiration can be effective in obtaining a preoperative diagnosis of SPENP, since the tumor has characteristic cytologic features. Also, use of clinical data, ultrasonography studies, computed tomography, magnetic resonance imaging, arteriography, and cytologic findings in the preoperative workup are important in obtaining an accurate diagnosis. Although potentially curable, late metastases and current inability to predict aggressive behavior by some tumors require lengthy follow-up.