We present the second reported case of a smooth muscle neoplasm involving the placental parenchyma. On gross examination, the tumor easily separated from the uterus and had a whorled cut surface with finger-like extensions into the villous parenchyma, very similar to the previously described case. The differential diagnosis included a primary smooth muscle tumor of the placenta (placental leiomyoma), a primary uterine neoplasm incorporated into the placenta, and a metastatic sarcoma. In this case, the infant was male, and the polymerase chain reaction technique demonstrated the presence of Y chromosome gene in the placental parenchyma and its absence in the placental neoplasm. Thus, this neoplasm, despite its gross appearance of a primary placental tumor, actually represented an incorporated benign uterine leiomyoma.