The aim of this study was to determine the profile of patients referred to a specialist oral medicine and oral pathology unit in Kuala Lumpur by reviewing clinical dental records received in Oral Pathology Diagnostic Service (OPDS) in Faculty of Dentistry, UKM from 2001 until 2010. A total of 547 archival biopsy clinical dental records were reviewed and analysed using SPSS version 17.0. Oral and maxillofacial diseases were frequently seen in female (1.3:1), young adults (30.0%) of Malay ethnicity (64.6%). Most of the acquired specimens were from dental specialists (n=451, 84.8%), particularly from oral and maxillofacial surgeons (OMFS) (n=349, 63.8%) compared to general dental practitioners (GDPs) (n=81, 14.8%). Almost all of the biopsy specimens were of soft tissue origin (n=462, 84.4%), derived from lining mucosa (n=197, 36.0%) and were biopsied excisionally (n=325, 59.4%) more often than by incisional biopsy (n=207, 37.8%). A large proportion of the oral and maxillofacial diseases were of reactive (n=188, 34.4%) and inflammatory (n=121, 22.1%) cause. Tumours are mainly benign (n=69, 12.6%) with only small cases are malignant (n=34, 6.2%). The most common histological diagnoses were accounted by mucocele (n=56, 10.2%), pyogenic granuloma (n=47, 8.6%), fibroepithelial polyp (n=38, 6.9%), radicular cyst (n=33, 6.0%) and periapical granuloma (n=29, 5.3%). This study characterizes the clinical profile of patients seen in our oral medicine and oral pathology unit. Present findings can be used as a reference to the clinicians and pathologists in effective patient management and organization in the future.