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In a female child presenting with vaginal bleeding or a mass protruding through the vagina, it is mandatory to perform an endoscopic and laparoscopic examination and biopsy to establish the correct diagnosis and precise extent of the lesion. An endodermal sinus tumor (EST) of the vagina is a rare, malignant germ cell tumor that occurs primarily in infants. Clearly, the vagina is a critical site for treatment, and the surgeon should seek to preserve the reproductive and sexual function, if possible. We report our experience of a case of EST of the vagina in a 9-month-old female with a history of metrorrhagia. We employed minimally invasive procedures for diagnosis and then, after the administration of platinum- based chemotherapy (PEB regimen), removed a residual lesion during follow-up. These procedures resulted in tumor regression. At follow-up, after two years, the patient is alive and disease-free.