In girls, endodermal sinus tumours are rare and malignant germinal tumours. They are highly lethal and it has previously been considered in cases with distant metastases at the time of diagnosis, that there is no satisfactory therapy. However, the rapid development during the last years of highly effective chemotherapeutic agents seems to have improved the survival rate. Already there are a few reports of surviving patients with metastatic disease. In our material of five girls with endodermal sinus tumour, one with metastatic disease has been treated according to a new protocol with combination therapy (cis-VAB). Four years after the operation she is alive and well without any sign of recurrence.