Due to improvements in cardiac surgery and perioperative care the number of adults with congenital heart disease is continuously growing. The perioperative and intensive care management of these patients is a challenge due to the variety of pathologies and surgical options as well as the complex pathophysiology. Many patients develop organ dysfunction with time and many require multiple cardiac operations as well as non-cardiac interventions during adulthood. While these patients are best treated in dedicated tertiary centers that provide a multidisciplinary expertise, basic knowledge of this population is important for everyone involved in acute medical care. This review will discuss some general aspects of adults with congenital heart disease such as pulmonary hypertension, Eisenmenger syndrome, cyanosis, pregnancy and perioperative care, with a special focus on the management of critically ill patients.