Exocrine pancreatic insufficiency is characterized by insufficient secretion of pancreatic enzymes with subsequent inability to maintain adequate digestion of food. Maldigestion may lead to malnutrition with associated various morbidities. Exocrine pancreatic insufficiency is also associated with a reduced quality of life and, in some studies, increased mortality. Exocrine pancreatic insufficiency may develop due to numerous causes and is often underdiagnosed and not adequately treated. Particularly in the early stages, diagnosis of exocrine pancreatic insufficiency may be difficult, as steatorrhea may be absent and a specific diagnostic test currently does not exist. Hence, it is crucial to recall the situations at risk for exocrine pancreatic insufficiency in order not to miss its diagnosis. In this article, we will provide a summary of the main causes of exocrine pancreatic insufficiency as well as its diagnosis and management.