Background: Ciliated cyst of the vulva is a variety of ciliated cutaneous cyst, which itself is a rare presentation and a very few cases has been reported.
Case: This case report shows the presentation of this cyst in a 12-year-old girl with early onset of puberty. The histopathology supports estrogen receptor and progesterone receptor positivity of the cyst lining, which supports the theory of Müllerian heterotopy during embryogenesis. This is the most popular theory on the development of this cyst. Its an uncommon presentation in children but a curable benign cyst.
Keywords: Ciliated cutaneous cyst; Ciliated cyst of vulva; Immunohistochemistry for estrogen and progesterone receptor; Müllerian heterotopy; Paramesonephric cyst; Urogenital sinus.
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