Endodermal sinus tumors(yolk sac tumors) are malignant germ cell tumors that usually arise in the gonads. We report the first case, to our knowledge, of endodermal sinus tumor of the scalp. The tumor was present in a developmental infant with an abnormal scalp and exhibited histopathologic and immunohistochemical features identical to those of endodermal sinus tumors of gonadal origin. The tumor resolved after surgical excision and chemotherapy, and the patient is at present alive. The purpose of this report is to add a differential diagnosis to neoplasms of the scalp in neonates and review approaches to the management of this rare clinical entity.