Abstract
Prader-Willi syndrome is the most common form of genetic obesity in children. The aim of our study was to analyse the progressive course of metabolically associated steatotic liver disease along with the development of pancreatic exocrine function deficiency in Prader-Willi syndrome. The presented clinical case is an example of the early development of metabolic steatotic liver disease in a child with Prader-Willi syndrome, complicated by liver fibrosis and exocrine pancreatic insufficiency. This clinical case is interesting because the patient developed signs of exocrine pancreatic insufficiency at an early age in the form of a decrease in pancreatic elastase in the stool - a consequence of fatty tissue replacement, i.e., the development of pancreatic steatosis. Therefore, the efforts of the treatment protocol are focused on a multidisciplinary approach including the examination of liver and pancreatic function. This allows for control of the progression of the disease, and reduces the risk of obesity-related complications. Despite the rarity of such cases, physicians should be alert in managing these patients.