Abstract Background Surgery is the only curative treatment for intraabdominal and retroperitoneal sarcoma (IaRS). Little is known about how to treat patients with recurrence. We here report the outcome in primary and recurrent sarcoma treated at the Sarcoma Center in Aarhus, Denmark. Methods All patients evaluated for IaRS from June 1998 to May 2008 were enrolled and data on symptoms, signs, means of diagnosis, extent of surgery, perioperative complications, mortality and long time survival were registered. Primary and first-recurrence sarcomas were analyzed separately. Results Sixty-five of 73 primary and 22 of 28 first-recurrence IaRS had surgery. Fifty-three (82%) and 11 (50%) patients achieved radical R0 resection. Age and radicality of surgery were independent predictors of death, while recurrence of sarcoma was not. Perioperative mortality was 2.3%. 5-year survival was 70.2% for primary and 51.8% for first-recurrent sarcomas. However, patients with radical surgery had 5-year survival of over 70% in both the primary and recurrent group. Conclusions The radicality of surgery is the most important prognostic factor. Patients with recurrence have an equally good prognosis as those with primary sarcoma if radicality is achieved and such surgery should not be considered only as a palliative effort.