Background: Hematological disorders may present with a number of non-specific orofacial manifestations that must be diagnosed and referred to specialists for treatment. Since the orofacial manifestations can be the first clinical presentation which indicates the presence of an underlying disease, it is important for dentists to be aware of these manifestations. The present study aims to evaluate the orofacial presentations of some hematological diseases in order to familiarize dentists with these manifestations.Methods: This descriptive cross-sectional study evaluated the oral conditions of patients recently hospitalized with histories of blood dyscrasia and bone marrow transplantation in Shiraz Nemazee Hospital during 2010-2011. From 50 patients, there were 33 (66%) males and 17 (34%) females. The age of participants ranged from 12-77 years of age.Results: Examined patients had the following manifestations: head and neck region lymphadenopathy (42%), hairy tongue, atrophy of the oral mucosa, ulcers, red and white lesions, Candida albicans infection and gingival lesions that included spontaneous gingival bleeding, gingival hypertrophy and ecchymosis, in addition to diffuse herpetic infections on the buccal mucosa and bony lesions. The most common blood dyscrasia in the study patients was acute myeloid leukemia (AML) (48%). Our findings determined that lymphadenopathy (42%) was the most common orofacial manifestation of hematological diseases.Conclusion:On occasion, dentists maybe the first medical professional to encounter hematological diseases. Since early detection of these disorders can increase patient survival, it is necessary for dentists to become completely familiar with these oral manifestations.