OBJECTIVE: To summarize the clinical experience of surgical treatment and chemotherapy for 18 cases of yolk sac tumor in children, and to analyze and discuss the relationship with prognosis. METHODS: We retrospectively analyzed the case data of 18 cases of yolk sac tumor in children admitted from April 1992 to December 2009 in our hospital. RESULTS: Among the 18 patients, there were 10 cases of testis, 3 cases of ovary, 4 cases of sacrococcygeal region and 1 case of abdomen. Seventeen patients (94.4%) were treated with surgical resection and regular chemotherapy. During the follow-up period of 1-2 years, no abnormality was found in the chest radiographs, no enlarged lymph nodes were seen in the retroperitoneum and pelvis by ultrasound and CT examination, and AFP was normal; in one case, the parents gave up the treatment because of the repeated recurrence of the tumor after the surgery. DISCUSSION: In the treatment of yolk sac tumor in children, the combination of surgery and chemotherapy can achieve a good therapeutic effect; AFP is of great significance as a tumor marker in the diagnosis and treatment of the disease.