Abstract

BackgroundSotos syndrome is one the most common overgrowth conditions, after Beckwith-Wiedemann syndrome. As with other overgrowth syndromes, Sotos syndrome can be associated with an increased risk of tumors. CaseWe describe a young girl with Sotos syndrome and ovarian fibromatosis with a new mutation not reported before in the literature. Summary and ConclusionDevelopment of ovarian tumor in Sotos syndrome has been poorly documented. Ovarian fibromatosis is a very rare non neoplastic disease. Management is guided by the benignity of the lesion and consists of surgical excision of the fibroma.

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