Objective To investigate the role of Notch signaling pathway in pancreas development and pancreatic cancer. Methods The related literatures were reviewed and analyzed. Results Notch signaling played a role early in development by maintaining pancreatic epithelial cells in a progenitor state and delaying their differentiation until timely appropriate. Notch signaling was reactivated in the initiation and progression of pancreatic cancer. Conclusion Notch signaling pathway plays an important role in the pancreas development. Sustained Notch signaling activity promotes the progression of pancreatic cancer, and may be one of major factors in the initiation of pancreatic cancer.