Background : Glycogen Storage Diseases are inherited inborn errors of carbohydrate metabolism which results in abnormal storage and metabolism of glycogen in the body, affecting mainly liver, cardiac and skeletal muscles. Patients with GSD are predisposed to development of hepatic adenomas, lesions which are inherently premalignant.

Methods : We present a case of a female teenage patient, diagnosed with incidental hepatocellular carcinoma (HCC), with underlying Glycogen Storage Disease (GSD). She was a 16 years old teenager, diagnosed with Glycogen Storage Disorder since age of 6. She had failure to thrive with clinical hepatomegaly, with subsequent liver biopsy confirming GSD. Other medical issues were secondary hyperparathyroidisim with recurrent issues of electrolyte imbalances. Diagnosis of multiple hepatic adenomas were made during follow up imaging by contrasted CT liver and MR liver primovist. Non-anatomical resection of the segment 8 liver lesion was performed in January 2023 after MDT. Surgery was complicated with intraoperative metabolic acidosis, therefore resection of other liver lesions were not performed. Eventful recovery complicated by prolonged ventilation and respiratory alkalosis. She was only discharged home a period of prolonged hospital stay.

Results : Histopathological exam reported as well differentiated hepatocellular carcinoma with intact liver capsule measuring 2 cm in size (pT1b), with clear resection margins of 1mm.

Conclusions : Association of GSD IV with hepatocellular adenoma has been shown, though most patients develop liver failure before the age of 5. These lesions occur in a background of non-cirrhotic liver; therefore, diagnosis and early detection remains a clinical difficulty.