These patients did not present with the characteristic clinical features and laboratory findings of phosphomannomutase-2-congenital disorders of glycosylation (PMM2-CDG, OMIM: #212065), which are the most common disease with variants in the PMM2 but presenting only with symptoms of ARPKD, hepatocytes, and hypoglycemia. The gene discussed is PMM2; the disease is congenital disorder of glycosylation.