Ameloblastomas are rare histologically benign, locally aggressive tumors arising from the oral ectoderm that occasionally reach a gigantic size. Giant ameloblastomas are a rarity these days with the advent of panoramic radiography in routine dental practice. Furthermore, the granular cell variant is an uncommon histological subtype of ameloblastoma where the central stellate reticulum like cells in tumor follicles is replaced by granular cells. Although granular cell ameloblastoma (GCA) is considered to be a destructive tumor with a high recurrence rate, the significance of granular cells in predicting its biologic behavior is debatable. However, we present a rare case of giant GCA of remarkable histomorphology showing extensive craniofacial involvement and dural extension that rendered a good prognosis following treatment.