Meigs syndrome is a rare complication associated with ovarian fibromas. Although ovarian fibromas are rare in children, they are common in women with Gorlin syndrome after puberty.A 14-year-old girl with Gorlin syndrome was admitted to our hospital for ablation of basal cell carcinoma. A chest x-ray revealed pleural effusion. Ultrasonography revealed bilateral multinodular ovarian masses. Meigs syndrome associated with ovarian fibromas was considered. A laparotomy revealed bilateral ovarian masses, which were resected. Microscopically, the masses were composed of mitotically active fibroma and areas resembling hemangiopericytoma and luteinized thecoma. The pleural effusion disappeared soon after the surgery.Physicians should consider the possibility that pleural effusion might precede the diagnosis of ovarian fibroma in patients with Gorlin syndrome.