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Hedgehog proteins are secreted molecules that bind to their cell surface receptors to elicit concentration dependent responses essential for numerous tissue patterning and cell differentiation events during embryogenesis. However, during early stages of pancreas organogenesis, hedgehog signaling has been shown to inhibit tissue morphogenesis and cell differentiation. By contrast, recent cell culture studies indicate that an active hedgehog pathway might be required for maintenance of adult endocrine cell functions. This review describes our current understanding of the requirement of hedgehog signaling during pancreas morphogenesis and cell differentiation and discusses how individual hedgehog genes might act at various stages to ensure proper pancreas development and organ function. (C) 2002 Elsevier Science Ireland Ltd. All rights reserved.