Midgut malrotation (MGM) refers to a broad spectrum of rotation and fixation anomalies of the gut during fetal development, which are clinically evident mostly early in life as newborn emergencies and rarely later as milder forms in adults. Most commonly, adult patients are asymptomatic throughout life or they are insidiously presenting unspecific symptoms for a significant period of time before the diagnosis is made. Less commonly, adult MGM cases are acutely complicated with challenging and life-threatening conditions, such as the obstructive midgut or right colon volvulus and the ileocolonic intussusception, which require prompt diagnosis, intensive resuscitation and emergent surgical intervention. Intentionally, high index of suspicion and awareness are essential, if bowel infarction is to be prevented and high morbidity and mortality reduced.