Children with severe neurodevelopmental impairment are at risk for recurrent hypothermia, defined as a temperature of less than 35 degrees C, as a result of hypothalamic dysfunction. Acute pancreatitis following hypothermia from environmental exposure or induced as medical therapy has been reported in adults. In this case series of 10 children (six males, four females) with severe neurodevelopmental impairment and associated hypothermia, five had an episode of acute pancreatitis. These five patients had documented hypothermia, an elevated lipase of greater than 1000U/L, and presenting symptoms of irritability or lethargy along with gastrointestinal symptoms such as feeding intolerance. Four of these five children had no other explanation for pancreatitis; the fifth had multiple gallstones. This case series identifies the risk of acute pancreatitis in children with central hypothermia. Monitoring for resolution upon establishment of euthermia can minimize unnecessary testing and cost.