Fanconi Bickel Syndrome (FBS) is a rare autosomal recessive disorder It is caused by homozygous or compound heterozygous mutations in GLUT2, the gene encoding facilitative glucose transporter in hepatocytes, pancreatic beta-cells, enterocytes, and renal tubular cells. To date, 112 patients have been recorded in the literature. Most patients exhibit the classic symptoms, including hepatomegaly due to glycogen buildup, glucose and galactose intolerance, fasting hypo-glycemia, tubular nephropathy and stunting.[1] Here we report a case of Fanconi Bickel syndrome with rare mutation which presented with hyperphosphatemia and no episode of hypo-glycemia. Diagnosis was made on the basis of clinical suspicion and confirmed by genome sequencing.