In the present case study, we present a female adolescent patient harboring a rare clinical presentation of spinal intradural extramedullary epidermoid cyst concomitant with congenital scoliosis. A 17-year-old female patient was admitted to the clinic with long-lasting neck pain. She was neurologically intact yet had cervicothoracic scoliosis. Cervical MRI and CT depicted a right C6 hemivertebra, fused to the lower endplate of the C5 vertebra. At the same vertebra level, she had an intradural extramedullary mass lesion anterior to the spinal cord. We planned to excise the mass lesion first. We used neuromonitoring during the surgery and made the surgery via posterior approach. We observed a pearl-like mass lesion anterolateral to the spinal cord. We excised the mass lesion with its capsule microsurgically via peace-meal route. She was neurologically stable following the surgery. Histopathological diagnosis was epidermoid cyst. Most of spinal inclusion cysts occur secondary to spinal dysraphism or iatrogenic inoculation. Isolated spinal inclusion cyst located anterior to the spinal cord concomitant with vertebral anomalies should be kept in mind before making proper surgical planning. Surgery is the modality of choice for spinal inclusion cyst and should be performed under the guidance of neuromonitoring, especially in cases with lesions located at higher spinal levels.