Background: Mucocele is expansile slowly growing benign cystic lesion that occurs as a result of occlusion of the ostia of the paranasal sinuses. It mostly involves the frontal sinus followed by ethmoid and rarely sphenoid and maxillary sinuses. Case presentation: Authors present a rare case of giant ethmoid mucocele in 34-year-old adult male presenting as nasal region mass with intracranial extension. Conclusions: Authors highlight that giant ethmoid mucoceles are rare and may have intracranial or extracranial extension resulting in craniofacial disfigurement which should be considered in patient presenting with nasal region mass.