Lipoblastomas are benign neoplasms of white adipose which usually occupy the trunk and limbs in pediatric patients. They can seldomly involve the head and neck, usually as a lateral cervical mass. Magnetic resonance imaging is the most useful modality for identifying these lesions. Treatment involves complete excision, and patients are followed with serial exams and/or imaging as recurrence is more common in the head and neck. Here we present an exceedingly rare case of retropharyngeal lipoblastoma in a two-year-old male causing severe obstructive sleep apnea which was identified during adenotonsillectomy. As this mass can be mistaken for other more common masses of the retropharyngeal space, we review the differential diagnoses, imaging, and histopathologic features of this neoplasm, which was responsible for upper airway obstruction in this case.