Complete agenesis of pancreas is a rare and lethal condition. Four cas
es have previously been reported in combination with other malformatio
ns, such as severe intrauterine growth retardation, hyperglycaemia and
meconium ileus. We report a case of pancreatic agenesis as a single a
nomaly. The child died when 48 h old with severe metabolic acidosis. T
he literature is surveyed and the pathogenesis is discussed.