Aim: To discuss a case of beta thalassemia major with its clinical intraoral, extraoral features and radiographic appearances and clinical management. Background: Thalassemia, a hereditary hemolytic anemia disease is the most widely distributed genetic disorder. It occurs due to a defect in the globin chain of hemoglobin. The more severe form, beta thalassemia major, presents with a diverse spectrum of clinical features caused by anemia, bone marrow hyperplasia, and blood transfusions. Case description: Here is a rare case presentation of a 4-yearold male patient suffering from thalassemia major, reported with pain and swelling in the upper front teeth region. The patient was under regular blood transfusion regimen. The patient was diagnosed with having severe early childhood caries and managed with full mouth rehabilitation. Conclusion: Thalassemia is characterized by diverse oral and dental features, a dentist should have complete knowledge of the thalassemia and its complications to treat the dental conditions effectively. Clinical significance: Caregivers of children suffering from systemic illness lacks the incentive to maintain oral health. As a result, children suffer from comorbid conditions such as early childhood caries. Furthermore, the systemic condition also affects their oral health where the necessity for early intervention and conservative management is indispensable.