Case records of 57 patients (50 female, 7 male) with sacrococcygeal teratoma who were treated at the Royal Children's Hospital in Melbourne between 1948 and 1986 were reviewed. There were 40 benign and 19 malignant tumors; two patients had malignant recurrence following excision of a benign tumor. The majority of the benign lesions were readily excised; 80% of these patients presented under the age of 6 months. In contrast, 16 of the 19 patients with malignant disease presented after 6 months of age and 12 of these died. Before 1975, malignant lesions were treated with surgery or irradiation, and in a few patients, single-agent chemotherapy. No patients survived. In 1970, intensive multiagent chemotherapy was introduced, with planned delayed surgical resection, with or without postoperative irradiation. Three of five patients treated between 1976 and 1980 survive disease-free and are almost certainly cured. Modern therapy is with cisplatin-containing regimens, and while initial responses in six patients with extensive disease are impressive, it is too early to evaluate the impact of these newer programs on cure.