We describe a case in which a continuous glucose monitor (CGM) was used during hospitalization to safely prevent severe hypoglycemia in a 10-month-old male with glycogen storage disease 1a undergoing placement of a gastrostomy tube. The child was admitted to the hospital for placement of a gastrostomy tube given increased severe hypoglycemic episodes at home secondary to worsening feeding aversion. During hospitalization, the CGM was monitored before, during and after surgery. Severe hypoglycemic episodes were avoided while hospitalized. The CGM device assisted with the management and titration of continuous dextrose infusion while the child was fasting. The CGM device improved patient and family centered outcomes by reducing the need for frequent finger stick point-of-care glucose checks and allowed the family and anesthesiologist to carefully monitor the child’s blood glucose continuously during the procedure.