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Histopathological characteristics and immunohistochemical findings are necessary for diagnosis. Resection of the lesion takes compression off the cord and therefore, is the primary line of management of this disease. Our case presentation serves as a reference when diagnosing and managing a patient of RDD. RDD with spinal involvement is rare and it is often a challenge to arrive at the correct diagnosis. Histopathological characteristics and immunohistochemical findings are necessary for diagnosis. Resection of the lesion takes compressi