In our cohort of 17 Korean patients with genetically confirmed Senior-Loken syndrome (SLS), four causative genes were identified: NPHP1 (35.3%), NPHP4 (29.4%), IQCB1 (29.4%), and SDCCAG8 (5.9%). The gene discussed is IQCB1; the disease is Sjogren-Larsson syndrome.