Neurofibromatosis is one of the phakomatosis (neurodermatoses) which are genetically determined hamartomatous or neoplastic diseases of the skin and nervous system. It is not a single entity, but a group of heterogeneous multisystemic neurocutaneous disorders involving both neuroectodermal and mesenchymal derivatives. Plexiform neurofibroma, a histopathologic variant of neurofibroma, is a hallmark of neurofibromatosis type I (NF-I). It is pathognomonic of NF-I and may be the first sign of neurofibromatosis. It often appears within the first 2 years of life or before adolescence and occurs in only 5% of NF-I cases. We present a case of intraosseous plexiform neurofibroma of the maxilla in a 2-year old female, which is rare, along with the oral manifestations and clinicopathological characteristics. Because NF-I is one of the most common genetic disorders and oral manifestations are common, knowledge of the variability of presentation in children is necessary for prompt diagnosis.