Intensive care unit-acquired weakness (ICUAW) is a severe complication in critically ill patients which has been increasingly recognized over the last two decades. By definition ICUAW is caused by distinct neuromuscular disorders, namely critical illness polyneuropathy (CIP) and critical illness myopathy (CIM). Both CIP and CIM can affect limb and respiratory muscles and thus complicate weaning from a ventilator, increase the length of stay in the intensive care unit and delay mobilization and physical rehabilitation. It is controversially discussed whether CIP and CIM are distinct entities or whether they just represent different organ manifestations with common pathomechanisms. These basic pathomechanisms, however, are complex and still not completely understood but metabolic, inflammatory and bioenergetic alterations seem to play a crucial role. In this respect several risk factors have recently been revealed: in addition to the administration of glucocorticoids and non-depolarizing muscle relaxants, sepsis and multi-organ failure per se as well as elevated levels of blood glucose and muscular immobilization have been shown to have a profound impact on the occurrence of CIP and CIM. For the diagnosis, careful physical and neurological examinations, electrophysiological testing and in rare cases nerve and muscle biopsies are recommended. Nevertheless, it appears to be difficult to clearly distinguish between CIM and CIP in a clinical setting. At present no specific therapy for these neuromuscular disorders has been established but recent data suggest that in addition to avoidance of risk factors early active mobilization of critically ill patients may be beneficial.