We present a family with TBS and variable clinical phenotypes. Patients in our family presented to the renal services with renal impairment or proteinuria. Two of these family members underwent a renal biopsy that showed focal segmental glomerulosclerosis (FSGS). Previous renal histology in TBS has been limited to autopsy reports. These have found focal disorganization of renal cortex, cystic renal tubules and dysplastic areas(3). This contrasts with our patients who presented slightly older and with distinctively different histological features. It is unclear if the FSGS was a secondary phenomenon due to the renal dysplasia but the patients that had a renal biopsy had normal sized kidneys on ultrasound and hence underwent the biopsy.