Lipomatous pseudohypertrophy (LPH) of the pancreas is an uncommon affection of exocrine pancreatic insufficiency. It is defined as substitution of the pancreatic exocrine gland by a large fat component. We report the case of a young patient with malabsorption syndrome (chronic diarrhoea and steatorrhoea), abnormal laboratory results and normal fibroscopy. Computed tomography revealed characteristic diffuse pancreatic abnormalities.