Introduction: Retroperitoneal teratoma is a rare congenital tumour, representing only 1% to 10% of primary retroperitoneal tumors in children, far behind ovarian and testicular locations. There is a clear female predominance with a sex ratio of 3.4/1. The purpose of this study is to review the clinical manifestations, the mode of revelation of retroperitoneal teratoma, the diagnostic means and the anatomopathologic aspect of retroperitoneal teratoma, then to show the contribution and the limits of the various imaging examinations: ultrasound, CT and MRI in the diagnosis of retroperitoneal teratoma. Observation: We report the case of a mature retroperitoneal teratoma discovered in a 10-month-old male infant. The diagnosis evoked by ultrasound and abdominal CT was confirmed by histological study of the excised specimen. The postoperative evolution after 3 months was favorable in particular with no signs of recurrence. Conclusion: Retroperitoneal teratoma is a rare congenital tumour. The complete imaging assessment including ultrasound and computed tomography is necessary preoperatively in order to make the diagnosis and to clearly determine the relationship with the various organs. The anatomopathological study confirms the diagnosis of retroperitoneal teratoma. Tumor excision must be radical to avoid recurrence, which readily occurs in a malignant form. Even if the tumor appears benign, postoperative monitoring based on clinical, biological and radiological examination is essential.
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