Neurofibroma is a benign peripheral nerve sheath tumor comprising variable mixture of Schwann cells, perineurial-like cells, and fibroblasts. Neurofibroma may occur as solitary lesion or as part of a generalised syndrome of neurofibromatosis or very rarely as multiple neurofibromas without any associated syndrome. There are two distinct variants of neurofibromatosis type I and type II. We present a case of neurofibroma of the hard palate associated with neurofibromatosis type I. The diagnosis of the lesion was made based on the clinical findings, family history, histopathology, and immunohistochemistry. Literature was reviewed and different types of neurofibroma, their incidence and frequency in the oral cavity, its association with neurofibromatosis, clinical manifestations, histopathologic characteristics, immunohistochemical analysis, behaviour, treatment, and recurrence are discussed.