Odontogenic keratocytes (OKC) is a benign cystic lesion of odontogenic origin with aggressive and infiltrative behavior. Maxillary sinus involvement is reported rare in OKC with <1% cases and could mimic symptoms of other Sino nasal neoplasm. Recurrence rate was high between 25-60% that attributed to surgical technique. We present a case of a 14year-old female presented with swelling at lateral side of left nose extended to the left cheek for 3 months. It was tender on palpation without pain nor hyperemic. There was no discharge from both nasal and oral cavity. Computerized tomography (CT) scan showed cystic mass with destruction of maxillary wall suspected as mucocele. Sub labial surgical approach was performed. Large cystic mass seen occupied left maxillary sinus with destruction of its anteroinferior wall. Displacement of several teeth roots was seen with canine root was attached in the lesion. Whole mass was completely enucleated without affected external appearance. Patient has been asymptomatic for 3 months follow up. Patient was referred to the dentist for teeth prosthetic treatment, however patient was felt satisfied with the cosmetic and functional result. Diagnosis of maxillary OKC is challenging. CT scan and/or Magnetic Resonance Imaging (MRI) were important to differentiate the cyst with other sin nasal neoplasms. Panoramic radiography can give important information related teeth involvement. Site and size of lesion, aggressive nature and high risk of recurrence in OKC contributed in choosing the best surgical technique. Surgical approach chosen should ensure the wide surgical field for complete resection while preserving cosmetic appearance.