Thalassemia, an inherited hemoglobin disorder, is the most common hemoglobinopathy in the world. Despite recent advances in the medical field to understand its pathophysiology, the management of thalassemia remains a complex process. Thalassemia patients are susceptible to various complications, including thromboembolic events. More commonly occurring thromboembolic complications include deep venous thrombosis (DVT), portal vein thrombosis, pulmonary embolism, and stroke. However, renal artery thrombosis has never been reported. In this report, we present a case of renal artery thrombosis in a thalassemia major patients.