Aim and Background: Beta thalassemia major is a hematologic autosomal recessive syndrome. Many anesthetic problems were noted in these patients like difficult intubation, coagulopathies and cardiomyopathies. In this case report, we describe a patient with Beta thalassemia major and intra cranial lipoma. Case report: A 36 year old woman, known case of Beta thalassemia major, was candidate for elective craniotomy for intracranial lipoma. She had marked deformities in maxillofacial bones and it caused difficult intubation. For this reason intubation was performed with fiber optic laryngoscope. Beta thalassemia major patients have difficult air way because of hypertrophic bone marrow due to marked facial and mandibular bone deformities and we must consider these problems for anesthesia management. Keywords: Intracranial lipoma, Beta thalassemia major, difficult intubation, Anesthesia management