Abstract
Congenital anomalies are not regularly seen in day-to-day practice. Crossed fused renal anomalies are even rarer. A 50-year-old female patient presented with right-sided pain in the abdomen with intermittent episodes of burning micturition. The patient was a known case of Mayer-Rokitansky-Kuster-Hauser syndrome. X-ray of the kidney, ureter, and bladder showed right-sided renal calculus. Computed tomography showed non-visualization of the left kidney in the left renal fossa but was crossed over to the right kidney with right renal pelvic calculus. A diagnosis of crossed fused kidney with calculus disease was made. Percutaneous nephrolithotomy was performed. With advancements in minimally invasive surgeries and imaging techniques, such large renal calculus in anomalous kidneys can be managed with good results.