Here, we report three novel splice site variants (c.68-2A > T, c.1311+4_1311+8del in ENG gene and c.526-6C > G in ACVRL1 gene) identified in three patients with a clinical diagnosis of HHT and ≥ 3 Curaçao criteria. Here, ACVRL1 is linked to hereditary hemorrhagic telangiectasia.