Background: Giant cell tumor (GCT) is a low frequency, benign neoplasm that generally produces lytic lesions. It mainly affects women in the third to fifth decade of life. Its most frequently reported location is in the temporal bone, especially the petrous portion, followed by the sphenoid. There are multiple lines of treatment; however, surgical management remains the standard treatment. Methods: An 18-year-old man presented with diplopia. Through imaging studies and biopsy, he was diagnosed with a GCT of the cranial base. The patient was managed surgically on two occasions and with complementary treatment with denosumab, which achieved complete remission and a total reduction in tumor volume. A systematic review of previously published studies was conducted. Results: We identified a total of 128 records through database searching from PubMed, Medline, and Scopus. The screening process left 23 records for detailed review. Upon further evaluation, nine non-research letters or commentaries were excluded, resulting in 15 full-text articles being included in the final review. Conclusions: GCTs have benign histological characteristics; however, the bony lytic destruction makes their management difficult. The main stray treatment modality continues to be surgery. However, as presented in our case supported with multiple studies adjuvant treatment with denosumab yields good responses. © 2025 Elsevier B.V., All rights reserved.