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Key Clinical Message Yolk sac tumors are rare and malignant germ cell tumors of the ovary occurring in children and young women. Fertility‐sparing surgical intervention with adjuvant chemotherapy has shown to improve prognosis. Abstract We present a case of a 14‐year‐old girl who presented with the complaints of lower abdominal pain and distention. Her tumor markers were increased, and radiological investigation suggested the diagnosis of malignant left ovarian mass. Histopathology confirmed the diagnosis of Yolk sac tumor. She was subsequently managed with fertility‐sparing surgery and adjuvant chemotherapy.