29-year-old female with chronic renal failure and a history of hemodialysis during 10 years showed localized jaw enlargement in the anterior mandible. She also revealed increased serum PTH level, osteosclerosis in her skull base and facial bone, chronic pain in the both knee joint, and gastrointestinal disorder which are symptoms of renal osteodystrophy. The patient was diagnosed hyperparathyroidism associated with chronic renal failure. Parathyroidectomy was performed, however, serum PTH level increased again after the parathyroidectomy, the affected area of the mandible was enlarged as the serum PTH level increased. The enlarged area seemed like as peripheral lesion clinically. We reported a case of localized jaw enlargement as oral manifestation in renal osteodystrophy.