Introduction and Importance: Cystic fibrosis (CF) is a widespread life-shortening recessive genetic disease and can present with sinus mucocele. Sinus mucocele is a rare condition, with limited prevalence data on unilateral proptosis. Case presentation: We present a case of a 19-month-old boy with CF who experienced worsening proptosis and exotropia in his right eye. A brain and orbit MRI revealed diffuse polypoid mucosal thickening, possible dense fungal deposit, deformity of the mid face, especially on the right side, with more prominent bulging of medical and inferior walls of the right lobe, a right ethmoidal mucocele causing ocular globe displacement, medial rectus compression, and optic nerve. An examination of the eye fundus showed disc edema and vascular congestion. Endoscopic sinus surgery successfully drained the mucocele, and treatment with antibiotics and corticosteroids led to symptom improvement and resolution of proptosis within three weeks. Clinical Discussion: Mucoceles represent an uncommon complication associated with CF in pediatric patients. Consequently, any child presenting with this issue should undergo evaluation for CF. Investigating this infrequent condition’s underlying mechanisms and consequences may improve treatment approaches and outcomes for those impacted. Conclusion: Sinus mucocele with unilateral proptosis in CF patients is uncommon, and endoscopic sinus surgery appears to be an effective cure for this complication, even in the pediatric population at high risk, like CF patients.
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