First report of mutation in δ-globin gene in an Iranian family

β-thalassemia with normal HbA2 and decreased MCV and MCH levels is a relatively rare form of β-thalassemia trait. Here, we describe a family with normal HbA2 and decreased levels of MCV and MCH. ARMS-PCR revealed IVSII-1 mutation in the β-globin gene in proband and her father. Direct sequencing of the delta-globin gene revealed a previously reported mutation called Troodos in the proband and her father (in cis with β-globin gene mutation). This is the first report of co-incidence of HbA2 Troodos and βIVSII-1 mutation. Coincidence of β and δ-globin gene mutation can cause problems in genetic counseling and prenatal diagnosis, due to masking the effect of β-globin gene mutation.