Hairy polyps, also known as dermoid polyps (DPs), are rare benign cystic lesions of bigerminal origin that may occur in several head and neck regions, including the oropharynx. Despite their benign histopathological nature, DPs may be life threatening, due to their upper airway location, and DPs represent one of the most unusual causes of respiratory distress during the neonatal period. In this paper, we describe a case of respiratory failure in a newborn with an oropharyngeal mass that was accidentally found during difficult intubation. Magnetic resonance imaging (MRI) detected a well-defined soft tissue pedunculated mass, arising from the left oropharynx wall, consistent with an oropharyngeal DP. The newborn had a prompt recovery after trans-oral mass removal. Our case underlines the importance of imaging in differential diagnosis of children’s respiratory distress, secondary to a variety of lesions within the region of the skull base or oropharynx. It allowed us to assess the origin of the lesion, as well as its relationship with the adjacent soft tissues, and to exclude intracranial extension, thus providing essential information for the surgical planning.