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Gastrointestinal manifestations are common in systemic lupus erythematosus (SLE). Intestinal pseudo-obstruction (IpO) is a rare, poorly understood and recently recognized gastrointestinal manifestation of SLE. We report a 26-year-old female with SLE for 3 years. 10 months ago, IpO was diagnosed first, and it was responded well to high-dose steroid therapy. After then, oral prednisolone and azathioprine were administered, but the patient had been lost to follow up until recurrence of IpO. She was admitted with diffuse abdominal pain, distension, frequent vomiting, and intermittent dysuria due to recurrent IpO accompanied with bilateral ureterohydronephrosis. Despite of high dose steroid therapy, her symptom and imaging findings were not improved. The patient was treated with pulses of cyclophosphamide, and then the patient's symptoms and signs were gradually subsided. Three weeks following cyclophosphamide therapy, she was able to eat without vomiting and following abdominal CT showed nearly complete resolution of diffuse intestinal distension and bilateral ureterohydronephrosis. High level of awareness of IpO in SLE and appropriate medical treatment is needed to prevent unnecessary surgical treatment. And if this complication is refractory to corticosteroid, active immunosuppressive therapy, such as cyclophosphamide, should be considered.