The correlation between hydrocephalus and spinal cord tumor was first described by Kyrieleis et. al. 1% concomitant presence was documented by Mirroni et.al. Moreover, the understanding of the pathophysiological association between these two conditions is still lacking. 11 years old girl presented in the outpatient department of Jinnah hospital Lahore with complaints of difficulty in walking for the last 2 years, numbness of right leg for the last one year, and severe bilateral sciatica for 2 months. On further inquiry, she told that she is also suffering from headaches and blurred vision for the last 6 months. Her neuroimaging revealed hydrocephalus and mixed density heterogeneously enhancing intradural lesion at the conus. She underwent ventriculoperitoneal shunting for hydrocephalus and laminoplasty followed by excision of spinal tumor reported being myxopapillary ependymoma grade II. The presence of headache along with signs & symptoms of raised intracranial pressure in these patients warrant cranial imaging. CSF diversion for hydrocephalus and gross total excision of the spinal lesion is the standard acceptable treatment.