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Neonatal sacrococcygeal teratoma is rare occurring in approximately one in 35,000 live births with a female predominance (3:1–4:1 ratio). It is an unusual tumor that develops before birth and arises from embryologically multipotent cells from the Hensen node, which is located in the coccyx. Sacrococcygeal teratomas generally present in two distinct fashions: newborns with large predominately external masses, which are noted in utero or at the time of delivery and are rarely malignant, and older infants and young children presenting with tumors predominately confined to the pelvis with a much higher rate of malignancy. Most sacrococcygeal teratomas are now diagnosed prenatally because of the routine obstetric ultrasonagraphy. An abnormally sized uterus is typically the first sign that a sacrococcygeal teratoma may be present. The increased-sized uterus can be due to a massive tumor or polyhydramnios. Less common presentations include maternal preeclampsia. Newborns generally present with the mass protruding from the sacral region. If detected prenatally, the neonate should be considered for abdominal delivery if the mass is greater than 5 cm in size. In addition, some develop in utero high-output cardiac failure resulting in fetal hydrops and may be candidates for in utero resection. When a prenatally diagnosed sacrococcygeal teratoma is associated with fetal hydrops, the tumor can become life-threatening to both mother and baby.A classification system has been devised by Altman et al. in 1974 and is still used today. Sacrococcygeal teratomas are categorized according to their location and severity. Type I tumors are predominately external. Type II tumors present with both internal and external components. Type III tumors are predominately hidden in the pelvis with a very small external portion. Type IV tumors are entirely hidden within the pelvis.The recommended treatment of a sacrococcygeal teratoma is complete removal of the tumor by surgery performed soon after the birth. Unfortunately, there is a significant recurrence rate of 10%–20% even with complete resection.Long-term follow-up is needed, as there is a risk of malignancy and reoccurrence. Postoperative follow-up is needed during the first several years of life as bowel and bladder functions can be affected. Children should be followed through potty training to ensure that bowel and bladder functions are normal.