Congenital neutropenias due to mutations in ELANE, SBDS or HAX1 or in the setting of glycogen storage disease (GSD) which is caused by <i>SLC37A4</i> mutation, often require prolonged granulocyte colony stimulating factor (G-CSF) therapy to prevent recurrent infections and hospital admission. Here, SBDS is linked to Glycogen storage disease due to glycogenin deficiency.