The incidence of calculus in augmented cystoplasty with Mitrofanoff on Clean Intermittent Catheterisation (CIC) is close to 30%. Mostly these patients present with supra pubic pain or urinary symptoms. The case reported here, presented with an unusual presentation of Acute Gastroenteritis and Septic Shock. He was having multiple vesical calculi in an augmented bladder with Mitrofanoff and was on regular CIC. Further, he underwent evaluation and after confirming the diagnosis. We performed Open Cystolithotomy and removed 540 gm of stones from the bladder. Post–operative period was uneventful, and the patient was discharged in hemodynamically stable condition. This type of presentation is unusual and has not been reported in the literature so far, high index of suspicion is required for early diagnosis of similar entity and to initiate appropriate management. Moreover, such complex cases should preferably be managed in a tertiary care center with a multidisciplinary team approach for better outcome.