Two neonates with intestinal obstruction and two children (aged 1 and 4 years) with severe constipation since birth are reported in whom stenosis of the distal rectum was found. In association with the rectal anomaly, three of them had a presacral tumour (teratoma in two, hamartoma in one) and all had a deformed sacrum. An embryological hypothesis to explain this association has been postulated by Currarino, after whom this triad has been named. Two patients were related (father and daughter). The role of hereditary factors in the occurrence of the syndrome has been reported before. Operative treatment of the rectal stenosis was necessary in all patients. Preoperative diverting colostomy was performed in three cases, followed by a posterior sagittal approach to excise the rectal stenosis and the presacral mass. In one case, persistent cerebrospinal fluid leakage required re-exploration for closure of a tear in a congenitally abnormal dural sac. The fourth patient had undergone a low anterior resection in the past via the abdominal route and needed rectal dilatation afterwards for some time. The final result in all patients appears satisfactory, although follow-up is short. Most cases of this triad have been reported in children but a number of patients have been diagnosed only as adults. Recognition of this triad should imply a careful search for neural crest malformations. Operative treatment to correct all soft tissue anomalies leads to good results.