Presacral teratomas are rare tumors derived from more than one embryonic germ layer and are usually diagnosed in infancy. It has been estimated that the incidence of presacral teratoma in children ranges from 1 in 30,000 to 1 in 43,000 live births. However, the diagnosis of these tumors in adults is extremely rare. H. Head et al. reviewed the world literature in 1975 and found only 71 reported cases. Since that time, an additional 14 cases have been reported, and only one of these was found in the United States. This study reports a case of an adult male who presented with recurrent infected pilonidal cysts that proved to be benign presacral teratoma at biopsy. The patient underwent resection by left hemisacrectomy and primary closure using a posterior approach and, since this procedure, has had no evidence of recurrence. The case is presented along with a review of the relevant literature.