Background: Proteus syndrome is a rare hamartomatous disorder characterized by the overgrowth of multiple tissues in a mosaic pattern. Tumors of genitourinary tract in Proteus syndrome are uncommon.
Case: We here report a 5-year-old girl with Proteus syndrome who developed a cystic mass in the pelvic cavity. The cyst was discovered by ultrasonographic examination and finally proved to be a unilateral ovarian dermoid cyst accompanied by an ipsilateral paratubal cyst by laparoscopic surgery.
Summary and conclusion: Proteus syndrome accompanied by an ovarian cyst is rare in girls. The possibilities of underlying ovarian cyst required for surgical intervention should be considered in Proteus syndrome.
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