We present the case of an intramedullary spinal cord tumor from C7 to T4, classified as a WHO grade I pilocytic astrocytoma, manifesting solely with isolated, acute hydrocephalus and a normal neurological exam in a 5-month-old infant. We discuss the common presenting symptoms of spinal cord tumors in the pediatric population and possible anatomical explanations for this unique presentation and offer recommendations for the management of isolated hydrocephalus in an infant.