Statement of Problem: The central nervous system and oral cavity have a close anatomical location. Recognition of the orofacial manifestations of neurological diseases is in direct relation with the responsibilities of a dentist.Purpose: The objective of this study was to familiarize dentists and even physicians with orofacial manifestations of related neurological diseases.Materials and Method: This cross- sectional study was performed on 1284 patients with known cases of neurological diseases referred from all over the Southern Provinces of Iran to Oral Medicine Department of Shiraz Dental School from 1997 to 2010. Orofacial manifestations of 34 different types of neurological diseases were classified in four main groups of cranial nerve disorders, main neurological disorders, neoplastic and tumoral neurological lesions and congenital anomalies of neurological disease. These groups were evaluated with respect to age, sex, signs and symptoms, clinical appearance and location of the lesions. Data were analyzed by using SPSS software version 15, by using relative frequency and frequency quotients.Results: Orofacial manifestations were shown in 532 patients of the first group (41.48%), 386 patients of the second group (30.11%), 243 patients of the third group (18.93%) and 123 patients of the fourth group (9.48%), respectively.Conclusion: According to the results of this 14 years study, cranial nerve disorders showed most of oral signs and symptoms followed by main neurological disorders, neoplastic and tumoral lesions, and congenital anomalies of neurological diseases respectively. So, dentists should be familiar with these manifestations to have a better recognition, diagnosis and correct decision upon treating these manifestations in such patients.