Pupil-involving oculomotor nerve palsy (ONP) is frequently associated with compressive lesion such as intracranial aneurysm originating from the posterior communicating arteries. Vascular variant of posterior intracranial circulation is regarded as an uncommon cause and association between these vascular variants and intracranial hypertension has not been reported. We present an 18-year-old girl with pupil-involving ONP combined with idiopathic intracranial hypertension who revealed compression of oculomotor nerve by a vascular variant of superior cerebellar artery (SCA). This is a rare case of an ONP attributed to compressive effect from an aberrant SCA affected by intracranial hypertension.