This is a case report of a female patient with multiple hepatocellular adenomas secondary to the glycogen storage disease 1a with asymptomatic and late adult-onset presentation. Even though the patient was taking a hormonal oral contraceptive, it was important to identify the genetic disorder and ensure adequate metabolic control which is considered a modifier of the adenoma formation and progression. It is also imperative that the patient gets monitored for the adenoma-to-hepatocellular carcinoma transformation.
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