Teratocarcinosarcoma is an extremely rare malignancy of the nasal cavity and paranasal sinuses. It exhibits both sarcomatous and carcinomatous components. Less than 100 cases are reported. It presents in adults with only two reported cases in infancy. Here we present a case of 3-week-old female with antenatally detected ocular mass. MRI revealed an exophytic right ocular mass (10 × 7.0 × 7.0 cm) with intracranial extension. The tumor consisted of malignant glands and mesenchymal elements of undifferentiated blastema-like cells and immature neuroepithelium. After an initial diagnosis and treatment for a Wilms tumor protocol, the mass showed no response. A second opinion rendered a diagnosis of sinonasal teratocarcinosarcoma. The patient underwent surgical resection and seven cycles of CNS ICE chemotherapy. A second debulking surgery revealed a very scant viable tumor with post-treatment changes. The patient is alive at 43 months on weekly vincristine maintenance. Molecular testing revealed a somatic CTNNB1 gene mutation. In conclusion, this is a rare and aggressive tumor which showed disease free survival beyond that reported in the literature with the appropriate use of multimodality therapy.

畸胎癌是一种极为罕见的鼻腔和鼻窦恶性肿瘤。它表现出肉瘤和癌成分。报告的病例不到100例。它出现在成人中，仅报告了两例婴儿期病例。在这里，我们介绍了一例 3 周大的女性产前检测到的眼部肿块。MRI 显示一个外生性右眼肿块 (10 × 7.0 × 7.0 cm)，伴有颅内延伸。肿瘤由恶性腺体和未分化胚泡样细胞的间充质成分和未成熟的神经上皮组成。在对 Wilms 肿瘤方案进行初步诊断和治疗后，肿块没有反应。第二种意见诊断为鼻窦畸胎癌。患者接受了手术切除和7个周期的CNS ICE化疗。第二次减瘤手术揭示了一个非常少的存活肿瘤，治疗后发生了变化。患者在每周长春新碱维持下存活 43 个月。分子检测显示体细胞CTNNB1基因突变。总之，这是一种罕见的侵袭性肿瘤，在适当使用多模式治疗的情况下，其无病生存期超过了文献报道的生存期。