A case of an incomplete Currarino triad is reported. The baby underwent an emergency laparotomy due to a life-threatening intestinal obstruction caused by severe rectal stenosis. During the posterosagittal anorectoplasty (PSARP), a presacral teratoma was identified and resected. The tumor recurred three times; she initially responded to chemotherapy, but nonetheless died at the age of 4 years. In cases with evidence of anorectal stenosis, a presacral mass should be suspected. PSARP is the best choice of treatment for both the anorectal anomaly and excision of the presacral mass. The presacral region should be followed up closely for recurrence of the tumor.