Pompe Disease is a rare and fatal genetic disorder of glycogen storage, characterized by low levels of the alpha glucosidase (GAA) enzyme. The symptoms and severity of the disease vary according to the amount of deficiency of GAA. Early onset or infantile Pompe disease is the most severe form of the condition, and is characterized by almost complete deficiency (<1% activity) of the enzyme. As of now, no specific treatment is available for Pompe disease. Treatment is mostly supportive and symptomatic. Clinical trials have been performed for an enzyme replacement therapy, and it shows promise for the immediate future.