Giant cell tumors (GCT) are generally benign, commonly affecting young adults, with a slight preponderance in females. They are locally aggressive with a high rate of local recurrence. Most of them are found in the epiphysis of long bones, making the base of the skull a rare site. We report the case of a 35-year-old female, who presented with neurological symptoms of headache and diplopia. On magnetic resonance imaging, a space-occupying lesion was discovered in the clivus. Histopathology was diagnostic of a GCT. GCT arising from the clivus is extremely uncommon, with about 15 cases published in the literature. The present case highlights the rarity of this tumor and contributes to the existing literature with analysis and evaluation of the management strategies and prognosis.