Case summary: The MRI of a 73 year old male patient with paraparesis, showed an intramedullary mass at the thoracic spinal cord extending from T6 to T8. Partial surgical removal was preformed and a biopsy was taken, followed by postoperative radiotherapy.

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Here we present a unique case of Harlequin syndrome without Horner syndrome after contralateral Th3 intradural tumor resection. Harlequin sign in our case presented probably to resection of sympathetic nerves while removing meningioma. Syndrome is rare in neurosurgical procedure, but we think that surgeons must be aware of it.