Background: Glycogen Storage Disease (GSD) Type 1b is a rareautosomal recessive condition The genetic defect leads to an absenceof the enzyme Glucose-6-Phosphatase, which is involved in both Glycogenolysisand Gluconeogenesis. Consequently, there is a tendencytowards life threatening Hypoglycaemia with a consequent requirementfor frequent oral intake. Associated features include Lactic Acidosis, Hyperuricaemiaand, Chronic Neutropenia resulting in oral ulceration and,periodontal disease. This may lead to problems with oral feeding and,potentiate life threatening Hypoglycaemia.Objectives: This case report describes an episode of Hypoglycaemia ina 23 year old GSD 1b patient following a recent course of antibiotics tocontrol her oral ulceration. This required in-patient management withintravenous dextrose and, frequent ingestion of corn starch.It serves to illustrate the bio-psycho-social issues of a young patient witha continual life threatening illness and, the effect her oral manifestationscan have in precipitating an acute episode.Methods: A Case report of a 23 year old patient with GSD 1b under longterm joint care of Endocrinology and Maxillofacial Surgery in HawkesBay District Health Board who presented in February of 2014. The reportdescribes the background of the Metabolic illness and its Oral Manifestations.Discussion: GSD 1b sufferers have a long term continued threatof Hypoglycaemia. Neutropenia secondary to the Metabolic defectcan precipitate severe Sepsis leading to Mortality. Further to this,Neutropenia can cause oral ulceration further compounding issuesof feeding. Antibiotic mouthrinses and, systemic antibiotic therapycan be helpful but may further affect gastrointestinal motility with thepossibility of impaired Glucose absorption. Sufferers therefore requireclose monitoring to manage their Oral Health.