Introduction: Fanconi-Bickel syndrome is an extremely rare disorder characterized by glycogen accumulation and generalized dysfunction of renal proximal tubules. It was first described by Fanconi and Bickel in 1949. This disease is caused by a defective monosaccharide transporter Glut2. Polyuria, metabolic acidosis, recurrent episodes of severe hypoglycemia, growth retardation, hepatomegaly, and rickets or osteomalacy are clinical consequences of this disease. However, a little information describing the dental and the orofacial signs and symptoms of this abnormality are available. Thus, the aim of this paper was to present the orofacial manifestations and dental management in a girl with Fanconi's syndrome. Case Report: In this paper, we report a dental findings in a case of 8-year-old girl with the Fanconi-Bickel syndrome who was referred to the Oral Medicine Department of La Rabta hospital (Tunisia). She suffered from many oral manifestations includes delayed development of dentition, opaque areas (hypocalcification), rapidly progressive periodontal disease and premature loss of temporary teeth, as well as general abnormalities. Conclusion: Through this observation, we conclude that the knowledge of craniofacial features and various dental abnormalities helps dentist to discover underlying systemic disease or syndrome. Thus reducing the morbidity of disease and improving the quality of life. Also, this case makes us thinking that an oral evaluation should be performed in all patients with Fanconi Bickel Syndrome.