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Relapsing polychondritis (RP) is very rare inflammatory disorder characterized by episodic, progressive, and destructive courses affecting cartilages. Renal involvement also is very rare presenting sign in these patients. It has seldom been described in children. We describe an 11-yearold girl with RP and complaint offrequent episodes of hematuria, and dysuria with fever and periorbital edema that auricular cartilage involvement appeared after renal involvement. Renal involvement in the setting of RP is mainly important, and need close observation.