Nasal septal lipoma in a child: Pai syndrome or not?

Lipoma is the most common soft tissue mass in adults but it is uncommon in children. Nasal septal lipoma is an even more rare entity. In this report, a 12-year-old girl was presented with congenital nasal septal lipoma. She had permanent partial nasal obstruction on her right side and disfigurement of her right upper lip. CT scan revealed a lipoma-like mass in the septum plus an additional incidental finding of an intracranial lipoma. Further workup with MRI demonstrated corpus callosum agenesis with interhemispheric lipoma. To remove the mass, septoplasty, nasal lipoma excision and nasal vestibule V-Y advancement flap were performed. To close the lip a rotational advancement flap was required. Our case seemed to be a variety of Pai syndrome. We recommend imaging of the central nervous system in newborns with congenital lipomas located in the craniofacial midline to rule out the Pai syndrome.