BACKGROUND/PURPOSE: The risk of malignant transformation of sacrococcygeal teratoma (SCT) and of presacral teratoma in Currarino syndrome (CS) may differ despite the similar position and appearance. METHODS: Malignant transformation and teratoma recurrence were assessed in a national retrospective comparative analysis of 205 SCT and 16 CS patients treated in one of the six pediatric surgical centers in the Netherlands between January 1981 and December 2010. RESULTS: The malignancy free survival of patients with SCT was lower than for patients with a presacral teratoma associated with CS (80% and 58% after one and two years in SCT versus 100% after two years in CS, p=0.017) CONCLUSIONS: In SCT, malignancy and recurrence risk are high. Therefore, early and complete resection is mandatory. Our data show that the risk of malignant transformation of a presacral teratoma in CS is small.