Yolk sac tumors (YSTs) of the ovary are rare neoplasm, accounting for less than 0.2% of all ovarian tumors. Although this malignancy is much rarer in children, pediatric cases are attributed to a poorer prognosis. Diagnosis of YST is often made by post-operative pathologic examination. The prevalent clinical presentations include fever, distended abdomen or asymptomatic abdominal mass. Yolk sac tumor rarely coexists with involvement of fallopian tube, and this entity is rare in children. The present research was conducted to present a rare case of ovarian yolk sac tumor in an 8-month-old female infant with involvement of fallopian tube. This report, presents a rare case of YST in terms of its clinical presentation, histopathology findings, imaging findings, diagnosis and treatment. The patient underwent surgical resection of the mass, followed by pathological examination, which confirmed a ovarian yolk sac tumor. To the best of our knowledge, this case is the youngest case of ovarian yolk sac tumor with the involvement of the fallopian tube reported in literature.