Background <p>Choroid plexus cysts are benign, typically asymptomatic cystic lesions found almost exclusively in the brain’s lateral ventricles, often noted during second-trimester fetal ultrasounds and usually resolving spontaneously before the 28th week of gestation. Spinal intramedullary choroid plexus cysts remain extremely rare, with only few cases documented in &#xa0;the&#xa0;literature to date. This case describes a 55-year-old man with thoracic spinal cord compression due to a choroid plexus cyst, highlighting a rare differential diagnosis for adult spinal cord lesions.</p> Case presentation <p>A 55-year-old Middle Eastern Syrian male with no remarkable medical history complained of chronic and persistent dorsal pain, and recently noted weakness in his lower extremities. A comprehensive neurological examination identified radicular pain radiating to the left lower limb along with evident muscular weakness in his right and left lower limbs. A thoracic magnetic resonance imaging identified a cystic lesion in the T5–T6 level, and the patient underwent cyst marsupialization and fenestration. Subsequent histopathological sampling and examination confirmed the diagnosis of choroid plexus cyst (refer to Graphical Abstract).</p> Conclusions <p>Choroid plexus cysts, though exceptionally rare in the spinal cord, should be considered in the differential diagnosis of intramedullary cystic lesions. Magnetic resonance imaging plays a crucial role in identifying these cysts and distinguishing them from other spinal pathologies. In symptomatic patients, surgical decompression may be warranted. Definitive diagnosis relies on histopathological analysis.</p> Graphical Abstract <p></p>