Glycogen Storage Disease Type I (GSD I), also known as von Gierke disease, is a genetic disorder that affects the body's ability to break down glycogen into glucose. This condition is caused by a deficiency in the enzyme glucose-6-phosphatase, which is essential for converting glycogen stored in the liver into usable energy. As a result, individuals with GSD I experience low blood sugar levels, especially during fasting. Symptoms of GSD I can include severe hypoglycemia, growth delays, and an enlarged liver. Management typically involves maintaining normal blood sugar levels through frequent meals and, in some cases, continuous glucose supplementation. Early diagnosis and treatment are crucial for improving quality of life and preventing complications.