<h4>Background</h4>Agenesis of the dorsal pancreas (ADP) is a rare disease, the pathogenic mechanism of which is partially related to variants of hepatocyte nuclear factor 1B (<i>HNF1B</i>) gene.<h4>Case presentation</h4>We report a case of ADP, which presented with acute ketoacidosis, hyperuricemia, and liver dysfunction. The gene discussed is HNF1B; the disease is hyperuricemia.