Yolk sac tumors (YSTs) are a type of malignant germ cell tumor that usually grow in the gonads. They are difficult to recognize at other sites outside the gonads, and no case has been reported involving the upper lip. The present study reported the case of a 13-month-old girl exhibiting an isolated YST occurring in the upper lip. The histology and elevation of α-fetoprotein were typical for a YST. The patient was cured following effective chemotherapy and surgery resection. After 36 months of follow-up, there was no sign of recurrence or metastasis. A total of 20 cases of primary YSTs of the head and neck extracranial region since 1997 were reviewed. The present study aims to inform the scientific community of the clinical and pathologic features of this patient.
Keywords: endodermal sinus tumor; germ cell tumor; upper lip; yolk sac tumor.