BACKGROUNDCholesterol granulomas are benign cystic lesions primarily found in the petrous apex of the temporal bone, often presenting diagnostic challenges due to their similarity to other lesions. Known for containing cholesterol-rich fluid, these granulomas can cause chronic inflammation and a range of symptoms, from mild headaches to severe neurological issues.CASE PRESENTATIONWe report a case of a 14-year-old Iranian female with recurrent flu-like symptoms, nasal congestion, dysarthria, and obstructive sleep apnea. Examination revealed a cystic mass in the nasopharynx, and imaging confirmed bilateral maxillary sinusitis along with a 37.5 × 15.3 mm cystic lesion in the nasopharyngeal area. The patient underwent Functional Endoscopic Sinus Surgery (FESS), successfully excising the mass.DISCUSSIONCholesterol granulomas typically arise in areas with compromised drainage, often linked to chronic inflammation. This case highlights the rarity of such granulomas in the nasopharynx, which can mimic various conditions. Histopathological analysis confirmed the diagnosis, underscoring the importance of microscopic evaluation. Differential diagnosis is critical in pediatric cases due to overlapping presentations with other conditions.CONCLUSIONThis case marks the first documented instance of a nasopharyngeal cholesterol granuloma in a pediatric patient, emphasizing the need for heightened awareness among healthcare providers. Further research is essential to elucidate the pathophysiology of this condition and develop standardized management strategies to improve patient outcomes.