Solid-pseudopapillary neoplasm of the pancreas is a relatively uncommon tumor. It typically affects young women, has nonspecific clinical and radiologic manifestations, and can be readily diagnosed by ultrasound-guided fine-needle aspiration and histopathologic evaluation. Histologic features characteristically show loosely cohesive, relatively uniform polygonal cells surrounding delicate capillary-sized blood vessels. Other features include cytoplasmic vacuolization, finely stippled chromatin, nuclear grooving, eosinophilic hyaline globules, and degenerative changes. Almost all solid-pseudopapillary neoplasms harbor mutations in the beta-catenin gene. They stain with beta-catenin, CD10, and focally with neuroendocrine markers. Although previously considered benign, this tumor is currently considered a low-grade malignant epithelial neoplasm with low metastatic rate and high overall survival. Most patients are cured by complete surgical excision. Despite the characterization of the morphologic and molecular features of this enigmatic neoplasm, more work is needed to uncover its cell of origin and true histogenesis.