Teratoma is a tumor that forms triploblastic tissues and the common sites of occurrence are sacrococcygeal lesions and the ovaries. The majority of cases are curable with surgical resection and the prognosis depends on the extent and histological scoring of the tumor. In the present study, we report a case of sacrococcygeal teratoma of a newborn showing features of a retina-like structure. A 29-year-old woman gave birth prematurely to an infant girl with sacrococcygeal teratoma. Surgical resection was performed 10 days after delivery. The tumor contained immature components as well as a retina-like structure. Several investigations, including immunohistochemical analysis, confirmed the similarities between the normal mouse retina and the retina-like structure of the tumor. The vascular arrangement and polarity surrounding the retina-like structure are unique and this is thought to be significant in the induction of structural differentiation. Our findings may provide insights into the matter of teratogenic activity in stem cell therapies for clinical applications.