Abstract
Adamantinomatous craniopharyngioma (ACP) is one of the two types of craniopharyngioma recognized by the World Health Organization (WHO), the other being papillary craniopharyngioma (PCP). These rare, benign tumours of the pituitary region are classified as Grade 1 central nervous system (CNS) tumours. ACP predominantly affects adolescents aged 5-15 years and adults over 50 years. It is usually located in the sellar and suprasellar regions. We present the case of an 18-year-old Malaysian female with a six-year history of persistent epistaxis and progressive nasal obstruction, an atypical presentation of ACP. This report highlights an entirely ectopic location of ACP in the sinonasal region. The tumour encompassed the left nasal cavity, the left anterior and posterior ethmoid sinuses, and the bilateral frontal sinuses. The unusual presentation of this tumour was detected with the aid of CT and MRI and confirmed by histopathological examination. In this case report, we discuss a rare presentation, an unusual location, and the strategies employed to overcome these challenges.