Pancreas divisum (PD), a congenital anatomic variant consisting of a separate pancreatic ductal system, is diagnosed in nearly 10% of patients for whom a successful pancreatogram was obtained. The relationship between PD and pancreatic disease is discussed since 1976. Some authors, reporting a higher incidence of pancreatitis in patients having two separate pancreatic ducts, proposed the concept of relative outflow obstruction of pancreatic juice through the accessory papilla. Based on the literature data and on our own experience, this review asserts that there is no definite evidence for PD being associated with a significantly risk of idiopathic pancreatitis. Discrepancies between epidemiological series could be explained by selection biases leading to an apparent association of PD and pancreatitis. An objective assessment of accessory papilla stenosis in patients with PD is not clearly available to give consistent results. Although controversies persist with regard to the actual abnormality of PD and the presence of stenosis into the accessory papilla, several endoscopic and/or surgical procedures have been proposed in an attempt to correct what is thought to be a stenosis of the orifice of the dorsal duct. More than 300 patients have been treated until now with variable and unpredictable results, these treatments are not devoid of complications. We conclude that PD should be considered as a frequent coincidental anatomic variant having no clinical significance in the great majority of patients and not requiring systematic further therapy.