We report a case of severe anemia caused by complex hereditary spherocytosis (HS) and X-linked sideroblastic anemia (XLSA) with two mutations in the spectrin beta (<i>SPTB)</i> and 5-aminolevulinic acid synthase (<i>ALAS2</i>) genes. The gene discussed is ALAS2; the disease is X-linked sideroblastic anemia 1.