Intramedullary spinal cord astrocytolna in infants is relatively uncommon, its occurrence is usually confined to the cervical and cervicothoracic regions. In this paper, we report on the case of a 4-monthold male infant with low grade holocord intramedullary spinal cord astrocytoma. He had developed progressive weakness of the lower extremities over a month period. Neurological examination revealed flaccid paraplegia as well as complete loss of all modalities of sensation below the T10 level. MRI revealed a large intramedullary mass which was found to be an intramedullary astrocytoma at surgery. This case report presents the clinical features, radiographic findings, and treatment and outcome for this patient together with a review of relevant literature.