Abdominal compartment syndrome (ACS) is a life-threatening entity that requires rapid recognition and treatment. This case report represents the first case report of ACS associated with the correction of a marked scoliosis. Of the many possible causes for respiratory compromise and cardiovascular collapse associated with major spine surgery, ACS should be considered, particularly in instances of profound spinal curvature correction. We present the case of an 8-year-old child with Duchenne muscular dystrophy (DMD) undergoing correction of a severe scoliotic curvature. Near the end of an otherwise unremarkable surgery, he developed severe respiratory compromise associated with respiratory acidosis, hypoxia, and hypotension in the face of a catastrophic decrease in lung compliance. After supine positioning and examination, he was discovered to have ACS, which was treated with laparotomy. Complete recovery occurred after 5 days. This case report should raise awareness of a rare, life threatening, but imminently treatable entity that can accompany scoliosis surgery. The description of this case should be particularly important for pediatric anesthesiologists and orthopedic surgeons who care for patients undergoing correction of marked scoliosis. We suggest possible mechanisms for the development of ACS in this setting that offer insights into the pathology of this entity, which could be useful in many other clinical situations where visceral venous drainage or bowel perfusion may be compromised.