Pancreas divisum (PD) is the most common developmental anatomic variant of pancreatic duct. The attention towards the PD has grown significantly since there are reports that this condition may cause acute relapsing pancreatitis, chronic pancreatitis and chronic abdominal pain syndrome. Furthermore, over the years, there have been multiple reports of PD associated with different types of tumors. There is evidence that PD can be associated with pancreatic tumors (up to 12.5% of cases). The golden standard for diagnosing PD is endoscopic retrograde cholangiopancreatography, but since it is an invasive procedure magnetic resonance cholangiopancreatography with secretin is a good alternative. In case the patient is symptomatic, endoscopic or surgical treatment should be performed. This review describes the key points of the pathophysiology, diagnostic modalities, risks of pancreatitis and tumors, as well as treatment options of PD.