Schinzel-Giedion syndrome which is associated with midfacial hypoplasia and coarse dysmorphic features is a multiple congenital malformation syndrome. It is believed that risk of malignancy may be a component of the anomaly. We report herein a case of a 16 months old boy with SGS associated with sacrococcygeal teratoma and cor-triatriatum dexter. Histopathology report of the excised sacrococcygeal mass showed no malignant foci. He was however lost to follow up in the second week post-operation.