Aim: This current report aimed at presenting a rare case of a large solitary intraoral neurilemmoma of the buccal vestibule followed by a brief review of literature of neurilemmoma published between 1997 and 2017. Background: The diagnosis of slow-growing intraoral lesions requires a thorough patient history and clinical examination. Despite the best efforts of even the most experienced oral medicine expert, a definitive diagnosis may sometimes only be reached through excisional biopsy and histopathological evaluation. Case description: To the best of our knowledge, there have been only two cases of neurilemmoma of the buccal vestibule reported in the literature till date and, looking at the size of the lesion, our case is the largest among the ones reported. Conclusion: In the present case, a rare presentation of solitary buccal vestibular neurilemmoma establishes the need for the consideration of a wide range of pathologies in the differential diagnosis. Clinical significance: Clinicians should include neurilemmoma in their differential diagnosis for asymptomatic, slow-growing intraoral growths and ensure complete excision to prevent tumor recurrence. The reader should be able to recognize the clinical features of neurilemmoma and describe the histopathological features and management of the condition.