| Summary: | <p>Abstract</p> <p>Introduction</p> <p>Encephalocele is a rare congenital abnormality of the central nervous system, where brain tissue protrudes from a defect in the skull. Some anomalies are associated with encephalocele. However, the association of spinal teratoma and encephalocele has not been reported in the English literature.</p> <p>Case presentation</p> <p>We report the case of an Iranian girl with a history of encephalocele surgery, who, at the age of four years, developed an intramedullary spinal teratoma, and discuss the pathogenesis of this association.</p> <p>Conclusion</p> <p>To the best of our knowledge, this is the first report of an association between encephalocele and spinal teratoma.</p>
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