Summary: Physiological proteinuria is common during pregnancy, but pathological proteinuria may also occur. Nephrotic range proteinuria most commonly occurs due to preeclampsia, but primary renal disease may also occur. We present a case of a woman who presented with nephrotic range proteinuria and nephrotic syndrome at an early stage of her second pregnancy. Due to proteinuria, haematuria, progressive symptoms and high need for medical treatment, renal biopsy was performed and she was noted to have focal segmental glomerulosclerosis (FSGS). She commenced immunosuppression but remained nephrotic during pregnancy. She delivered a healthy baby boy at 35 weeks gestation. Postpartum she remained nephrotic, and developed further complications requiring renal replacement therapy. Despite a successful pregnancy, this case of FSGS highlights that despite aggressive treatment FSGS in pregnancy is often associated with adverse renal outcome. We discuss investigations, including renal biopsy and empiric treatment options in this report.