Background: Presacral tailgut cysts are uncommon and few data exist on the outcomes following surgery. Methods: Patients undergoing tailgut cyst resection at the Mayo Clinic between 1985 and 2008 were analysed retrospectively. Demographic data, clinicopathological features, operative details, postoperative complications and recurrence were reviewed. Results: Thirty-one patients were identified (28 women), with a median age of 52 years. Seventeen patients were symptomatic and 28 had a palpable mass on digital rectal examination. Median cyst diameter was 4.4 cm. Four patients had a fistula to the rectum. Complete cyst excision was achieved in all patients; eight underwent distal sacral resection or coccygectomy. Postoperative complications occurred in eight patients but without 30-day mortality. Malignant transformation was present in four patients: adenocarcinoma in three and carcinoid in one. The cyst recurred in one patient after surgery for a benign lesion. Conclusion: Presacral tailgut cysts should be removed due to the risk of malignant transformation.