Problem To report the rare occurrence of a yolk sac tumor of the nose and nasopharynx presenting as acute sinusitis and bilateral complete blindness. Methods The clinical presentation, pathologic features, and management of this 2-year-old infant girl is reviewed. We also performed a medical literature search in English using PUBMED and OVID databases. We then analyzed the literature with respect to clinical presentation, manifestations and therapies for other extra-gonadal yolk sac tumors presenting in the head and neck. Results The patient was treated with 4 courses of high dose cis-platinum, etoposide, bleomycin repeated every 3 weeks along with surgery. She had initial mild improvement in visual perception along with shrinkage of her tumor. The yolk sac malignancies of the head and neck are often, large, aggressive lesions on presentation that once treated have a tendency to recur. Conclusion Yolk sac tumors (endodermal sinus tumors) represent 3–5% of pediatric malignancies. They are rare malignancies in the head and neck, of germ cell origin. Successful treatment usually requires a combination of chemotherapy and surgical extirpation followed by postoperative chemotherapy. Significance Recurrent sinusitis and nasal obstruction in children should be evaluated carefully and with a high level of suspicion for rare nasal and nasopharyngeal malignancies. Early diagnosis and prompt treatment may alleviate many of the presenting symptoms and prolong life.