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Congenital teratomas occur in extragonadal locations, the commonest site being the sacrococcygeal region. This report describes a rare case of antenatally detected, large, immature retroperitoneal teratoma. The diagnostic and therapeutic challenges of dealing with such a case have been discussed and the relevant literature reviewed. The recurrence of the tumour after gross surgical removal indicates a definitive role of administering chemotherapy in such a case.