Serological testing for M-type anti-phospholipase A2 receptor antibodies (antiPLA2R Ab) has abrogated the need for kidney biopsy to diagnose membranous nephropathy in the appropriate clinical setting. We report a case of a 63-year-old hypertensive male who presented with nephrotic syndrome associated with autosomal dominant polycystic kidney disease which was effectively diagnosed with the use of antiPLA2R Ab test. He achieved complete remission upon treatment with Rituximab. Hitherto, all anecdotal case reports of this uncommon association were diagnosed only by kidney biopsy. We highlight the usefulness of PLA2RAb especially in such cases of difficult-to-biopsy kidneys.