This article reports on a newborn with a scrotal space-occupying lesion and histologically proven granulosa cell tumor of the testes. A massive scrotal space-occupying lesion was diagnosed in a full-term male neonate with a birth weight of 2,260 g and body length of 45 cm. Sonography of the right testicle revealed a mass 6x6x4 cm in size which was multiply septate and cystic. The testicular parenchyma was poorly defined with a Swiss cheese appearance. Chemical analysis of blood serum evidenced normal levels of testosterone, beta-HCG, and inhibin B as well as physiologically a clearly elevated alpha-fetoprotein level at 35,350 ng/ml. Based on the clinical and sonographic findings of the right testicle, inguinal surgical exposure of the testes was undertaken. Since there was hardly any testicular parenchyma, we decided to perform high inguinal ablation of the right testicle. This case report confirms that congenital granulosa cell tumors usually exhibit benign behavior during the neonatal period.