Background: We report a rare condition of Pneumatosis cystoides intestinalis with hepatic portal venous gas in a 63-year-old man with good prognosis.Case Report: A 63-year-old man presented with fever, shaking chills, headache and malaise from three days before admission. The blood pressure was 130/80 mmHg; body temperature was 39.2° centigrade; respiratory rate was 18 per minute, and pulse rate was100 per minute. Physical examination revealed a mildly icteric sclera, Leukocyte count was 3800 per µl, hematocrit was 38%, and in urinalysis Bilirubin was +3 positive total Bilirubin was 5.7 mg/dL, Bilirubin direct: 3.5 mg. Blood culture was positive for E. coli. The patient was given high-pressure oxygen, and antibiotic therapy. fever was diminished and clinical status was better. The patient was discharged one week after therapy, while CT-scan showed no air in the portal venous system and intestinal gas-filled cysts was decreased.Conclusions: Many studies showed that Pneumatosis cystoides intestinalis alone can be seen in benign conditions. But Pneumatosis cystoides intestinalis with hepatic portal venous gas are signs of severe problems in abdomen with poor prognosis. We report a rare case of this condition with good outcome.

Keywords: Pneumatosis cystoides intestinalis, hepatic portal venous gas