Objective: Inherited metabolic diseases, including glycogen storage
disease (GSD), are frequently seen in Iranians due to the high
consanguinity rate, but the distribution of various types of GSD is
unknown. Hypoglycemia, hepatomegaly, growth retardation,
hyperlipidemia, hyperlactacidemia, and hyperuricemia are the common
features of GSD type 1, inflammatory bowel disease (IBD)-like colitis
is a known entity in GSD. Case presentation: We report a female patient
with type Ia GSD (GSD Ia) who was followed-up for more than 17 years.
GSD Ia was diagnosed based on biochemical tests and the pathology from
a liver biopsy as she was 3 years old. Conclusion: With ageing, more
and more complications will develop, of which those related to liver
adenomas are likely to be major causes of morbidity and mortality
