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Syringomyelia is a chronic progressive disease of the spinal cord. In symptomatic patients, bilateral sensory motor signs and symptoms prevail, moreover they can develop gastrointestinal disorders, although few studies have succeeded in explaining this correlation so far. We report a case of a 67-year-old woman with a history of pain in the back-lumbar spine and lower limbs, paresthesia and urinary incontinence. MRI revealed syringomyelia, extended from T3 to the medullary cone. Neurological picture was worsened by progressive and increasingly debilitating gastrointestinal symptoms refractory to dietary changes and medical treatment. Blood tests, gastrointestinal investigations and imaging were all normal apart from scintigraphy which confirmed delayed gastric emptying. The neurological symptoms disappeared after removal of an hemangioblastoma of the medullary cone. The persistent gastroparesis was treated by total gastrectomy with complete resolution of the patient's gastrointestinal symptoms., Highlights • Syringomyelia is a spinal disorder rarely associated with gastrointestinal dysfunction. • We present a case of refractory gastroparesis in a female patient with syringomyelia. • Surgery may be indicated if dietary changes and medical management fails. • We performed a total gastrectomy with improvement in the patient's quality of life.