Glycogen storage disease (GSD) type IIIa (Forbes-Cori disease) can be associated with severe liver disease. A patient with GSD type IIIa may therefore be a potential candidate for liver transplantation. Progressive myopathy makes uncertain the outcome of the patient and the transplant. Herein, we report on the good results of liver transplantation up to 28 months after the transplantation in a 40-year-old man with liver cirrhosis and significant muscle weakness due to GSD type IIIa.