Acute pancreatitis associated with varicella-zoster infection is a rare event, particularly in immunocompetent children. We report on a case of acute pancreatitis in a 6-year old girl presenting with acute abdominal pain less than 72 hours after the onset of a typical vesicular rash. The diagnosis was confirmed through hyperamylasaemia, ultrasonographic findings of oedematous pancreatitis and acute seroconversion to varicella-zoster virus, after excluding more common causes of acute pancreatitis. Conservative treatment was initiated, with complete resolution of symptoms and absence of local or systemic complications on follow up. A review of the literature revealed only three previously reported cases, with very different outcomes, highlighting the need to consider this potentially fatal complication in every child presenting with acute abdominal pain during the course of varicella disease.