Glycogen storage diseases (GSD) are genetic metabolic disorders of glycogen metabolism. There are >15 types based on the enzyme deficiency and the affected organ. Glycogen storage disease Type Ib is the only type associated with neutropenia and periodontitis. This type is caused by a deficiency of glucose-6-phosphate (G6P) translocase which prevents the transport of G6P across the endoplasmic reticulum. As a result, glycogen cannot be metabolized into glucose with its subsequent accumulation in tissues. The affected organs involved in Type Ib are the liver, kidney, and intestine. A 5-year-old Jordanian boy from a consanguineous family referred to the periodontal clinic in February 2014 with an established diagnosis of GSD-Ib. The systemic manifestations include hepatomegaly, hypoglycemia, hyperprolactenemia, inflammatory bowel disease, osteoporosis, and neutropenia. Oral manifestations include severe gingival inflammation and recurrent oral ulceration disease. The clinical signs and symptoms of periodontal disease in GSD Type Ib are similar to those found in patients diagnosed with neutropenia. Future studies are needed to clarify whether severe generalized inflammation of the gingiva in children is part of the GSD Type Ib or is a separate entity caused by neutrophil dysfunction.