Spinal ependymomas are the most common intramedullary spinal tumor, with a large proportion containing a small intratumoral cyst. Although the signal intensity varies, spinal ependymomas are generally well-demarcated, are not associated with a pre-syrinx, and do not extend above the foramen magnum. Our case demonstrates unique radiographic findings of a cervical ependymoma with a staged approach to diagnosis and resection. The patient is a 19-year-old female who presented with a three-year history of neck pain, progressive arm and leg weakness, falls, and functional decline. MRI revealed an expansile dorsal and centrally located T2 hypointense cervical lesion with a large intratumoral cyst extending from the foramen magnum to the C7 pedicle. Contrasted T1 scans showed an irregular enhancement pattern along the superior tumoral border down to the C3 pedicle. She underwent a C1 laminectomy for open biopsy and cysto-subarachnoid shunt. Postoperative MRI revealed a well-demarcated enhancing mass extending from the foramen magnum to C2. Pathology revealed Grade II ependymoma. She underwent an occipital to C3 laminectomy with gross total resection. Postoperatively she experienced weakness and orthostatic hypotension that improved remarkably upon discharge. Initial imaging was concerning for a higher-grade tumor, with holocervical cord involvement and cervical kyphosis. Given concern for grade and possible extensive C1-7 laminectomy and fusion for resection, a smaller surgery involving drainage of the cyst and biopsy was performed. Postoperative MRI revealed regression of the pre-syrinx, improved tumoral definition, and improvement of cervical kyphosis. This staged approach spared the patient unnecessary surgical intervention such as extensive laminectomy and fusion. We conclude that in cases of a large intratumoral cyst in an extensive intramedullary spinal cord lesion, open biopsy and drainage followed by resection in a staged fashion should be considered. Radiographic changes from the first procedure may affect the surgical approach for ultimate resection.