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Intramedullary glioependymal cysts are rare congenital lesions of the spinal cord. Their association with another congenital lesion, i.e., tethered cord, is rarer still.We present a case of intramedullary glioependymal cyst with a filar lipoma in an infant. The clinical features, terminology and pathogenesis are discussed. Many varieties of spinal cysts have been described. It is now believed that simple ciliated or goblet-containing intracranial and intraspinal cysts are endodermal rather than glioependymal in origin. The diagnostic criteria, too, have been defined. Although many of these lesions are developmental in origin their association with other congenital anomalies is rare.