Background <p>Chordomas are rare, slow-growing but locally aggressive tumors that most often arise in the clivus and sacrum. Their occurrence within the mobile spine is uncommon, and intramedullary cervical chordomas are exceptionally rare, with very few reported cases. This report adds to the limited literature by describing an unusual intramedullary cervical presentation in a young patient, highlighting the importance of broad differential diagnosis in acute spinal cord compression syndromes.</p> Case presentation <p>A 14-year-old female presented with rapidly progressive weakness of both lower limbs, which extended to the upper limbs within four days, accompanied by bladder dysfunction of the upper motor neuron type. On neurological examination, she was found to have spastic quadriparesis with paraplegia. Magnetic resonance imaging of the cervical spine revealed an intramedullary cystic lesion at the C5 to C7 levels with a hemorrhagic component. The patient underwent surgical excision, which demonstrated a pseudocapsulated lesion with a nodular component. Histopathological examination and immunohistochemical analysis confirmed the diagnosis of chordoma.</p> Conclusions <p>Intramedullary cervical chordomas are extremely rare and are often not considered or misdiagnosed clinically and radiologically in the differential diagnosis of acute spinal cord compression, especially in pediatric patients. This case underscores the importance of maintaining a wide diagnostic perspective in rapidly progressive myelopathy. Early recognition through imaging and confirmatory histopathology enables timely surgical intervention, which remains the cornerstone of management and may improve neurological outcomes.</p>