A 26-year-old woman who had been on hemodialysis for 7 years presented with a gradually swelling palatal lesion causing difficulty in phonation, dysphagia, and splaying of the maxillary teeth (Fig. 1A). Her PTH levels had been very high for 6 years. At this presentation, laboratory results showed: parathyroid hormone (PTH) level > 3,000 pg/mL, phosphorus level 6 mg/dL, alkaline phosphatase level 1,416 U/L, and normal calcium level (9 mg/dL). Computed tomography showed expansile lesions over bilateral mandibular bodies and palate with ground-glass appearance and mild osteolytic change of the left lateral sphenoid ridge (Fig. 1B). An incisional biopsy showed proliferation of fibrous tissue with a misshapen trabecular bone, scant osteoblastic rimming and mild increase in osteoclastic infiltration, compatible with fibrous dysplasia or brown tumor. Guanine nucleotide-binding protein/alpha-subunit (GNAS) exon 8 DNA sequencing showed substitution of arginine 201 with serine (R201S), confirming the diagnosis of fibrous dysplasia. She received subtotal parathyroidectomy and deceased kidney transplantation 2 months later. To correct the facial deformity, she received partial maxillectomy, resection of the maxillary tumor followed by reconstruction and tooth replantation nearly 1.5 years later. After nearly 2 years of follow-up, she had satisfactory function and aesthetic appearance (Fig. 2).