Granular cell tumor is an uncommon soft tissue tumor with controversial histiogenesis that can affect any organ of the body including orofacial region. It is mostly benign neoplasm. Neumann’s tumor and Abrikossoff’s tumor are rare entities with different histiogenesis with varied clinico-pathological presentations. The etiology of the Granular cell tumor remains unclear due to its muscular, connective or neural tissue origin. It is a matter of long debate and diagnostic dilemma. We are presenting this case report of congenital granular cell tumor in a 2 days old baby over an anterior maxillary alveolar ridge; diagnosed on histopathology and immunohistochemistry. The aim of this report is to highlight the rarity of congenital granular cell tumor and its difference with other granular cell tumours.