Abstract Background Isolated onset of intracranial hypertension due to spinal cord tumor is rare, thus, easily leading to misdiagnosis and delay in effective treatment. Case presentation Herein, we describe a 45-year-old female patient who manifested isolated symptoms and signs of intracranial hypertension and whose condition was initially diagnosed as idiopathic intracranial hypertension and transverse sinus stenosis. The patient received a stent implantation; however, no improvements were observed. One year later her symptoms exacerbated, and during rehospitalization a spinal imaging examination revealed a lumbar tumor. Pathologic evaluation confirmed schwannoma, and tumor resection significantly improved her symptoms, except for poor vision. Conclusions Space-occupying lesions of the spine should be considered in the differential diagnosis of idiopathic intracranial hypertension, even in the absence of spine-localized signs or symptoms.