Medullary sponge kidney (MSK) is usually a benign process which may remain asymptomatic and undetected for life. Incomplete distal renal tubular acidosis (RTA) may be found in as many as 30% to 40% of these patients. Clinical presentation usually occurs after 20 years of age. It may present with renal colic, recurrent urinary tract infection (UTI) and gross hematuria. We are presenting a case of 30 year old female who presented with recurrent urinary tract infection, underwent multiple surgeries for renal stone and due to insufficient follow up developed complications related to forgotten ureteric stent. She finally landed up as poorly functioning symptomatic right kidney and non-functioning left kidney for which nephrectomy was done. This case highlights the importance of earlier diagnosis, proper management and frequent follow up to preserve renal function in a case of MSK.