Teratomas are neoplasms of the embryonic tissues that typically arise in the gonadal and sacrococcygeal regions. Primary adrenal teratoma are extremely rare and only few cases were published in literature. Teratomas contain more than one embryonic germ cell layer, mostly elements derived from ectoderm and least frequently from endoderm. Though these tumors are mostly benign, malignant transformation may occur. Treatment includes surgical removal. We report a rare case of a primary mature retroperitoneal teratoma in an infant with liver metastasis. Imaging modality CT and MRI were useful in diagnosis. The diagnostic and therapeutic challenges of dealing with such a case have been discussed and the literature reviewed.