Abstract: Objective: To analyze and summarize the clinical characteristics of patients with spontaneous hemorrhage of hepatic adenoma in glycogen storage disease type Ia. Methods: Reporting 1 case in our hospital and making a summary about general situation, category, etiology, diagnosis and treatment of the hemorrhage of hepatic adenoma with glycogen storage disease type Ia through checking literatures. Results: The patient was a 27 year old male who had been diagnosed as glycogen storage disease for 14 years, as well as was first found hepatic adenoma at the age of 17. He once was diagnosed as intra-adenoma bleeding with persistent abdominal pain and dizziness and was underwent selective hepatic artery embolization at the age of 22. Hepatic adenoma in glycogen storage disease type Ia generally appeared at the age of puberty. One common complication of this disease was hemorrhage of hepatic adenoma, which can be found by ultrasonography and CT. Treatments included observation, selective hepatic artery embolization, radiofrequency ablation, surgical resection and liver transplantation. Conclusion: Glycogen storage disease type Ia is an autosomal recessive genetic disease, some patients may be secondary to hepatic adenoma, the radiological examination can be helpful to detect hepatic adenoma. Then appropriate intervention can be useful to improve the quality of life and prognosis.

Key words: Glycogen storage disease type Ia, Hepatic adenoma, hemorrhage