Thalassaemia, one of the most common genetic disorders, often causes serious medical, social, and psychological problems. Beta thalassaemia major is a life-threatening disorder that presents with a vast variability in the systemic signs and symptoms. In addition, orofacial and dental tissues are also affected. The common orofacial features among thalassaemic patients include: frontal bossing, skeletal overgrowth with characteristic appearances known as chipmunk faces, upper lip retraction, protrusion of pre maxilla bone associated with alveolar enlargement that causes malocclusion in the dentition with the clinical appearance of protrusion, flaring, spacing of anterior teeth and anterior open bite. The oral mucosa appears pale or a lemon yellow colour due to deposition of bilirubin pigmentation and anaemia. Sometimes the gingival colour tends to be dark, caused by high ferritin level in the blood. Current reports show a significant improvement in thalassaemia major patients’ survival rates. With increased life expectancy, the need for improved oral healthcare is very important to ensure a high quality of life for this patient population. This paper reviews the literatures and discusses briefly the dento-facial manifestations, radiographic features, dental caries, periodontal and soft tissue conditions related to beta thalassaemia major as well as dental management and considerations of thalassaemia patients.