Neurilemmoma, also known as Schwannoma, is a benign, slow-growing, encapsulated neoplasm that arises from Schwann cells of the peripheral nerve sheath. Although it is extremely rare, it affects the head and neck region in roughly 25–45% of all cases. Intraorally, the tongue is the most common site and is rarely involves vestibular mucosa. Here, we report a rare case of Schwannoma of vestibular mucosa in a 13-year-old girl and contribute a review to the current literature. Clinically, based on age, site, and appearance, a diagnosis of a benign tumour such as lipoma, fibroma, neurofibroma, and benign lesions of salivary glands was made. An excisional biopsy showed solely Antoni type A tissue with central acellular eosinophilic Verocay bodies surrounded by spindle-shaped neurilemma cells arranged compactly with wavy, twisted nuclei arranged in a palisaded manner. The patient was recurrence-free after 6 months. Hence, this case is of interest due to its rarity in terms of age, site, and histopathology (Antoni type A neurilemmoma) for a presumed initial diagnosis. Here, we also hypothesize regarding the type of growth pattern in the earlier diagnosed cases.