Purpose: To identify adult multicystic calcified dysplastic kidneys (AMCDK) with contralateral ureteral abnormalities mimicking urinary tuberculosis and to evaluate the feasibility and efficacy of retroperitoneoscopic extirpation in their management. Patients and Methods: We retrospectively identified a group of adult patients who were referred to us as having unilateral nonfunctioning kidney containing calcified cystic masses with a contralateral normally functioning kidney along with segmental dilation of ureter. Two patients had histories of treatment elsewhere with antitubercular drugs on the basis of imaging studies, before being referred to our center for retroperitoneoscopic nephrectomy with a diagnosis of nonfunctioning left kidneys and urinary tuberculosis. The other two cases with similar findings on imaging studies were detected incidentally while the patients were undergoing investigations for vague abdominal symptoms. Results: All these patients had AMCDK on the left side and a contralateral normally functioning kidney with ureteral abnormality. Retroperitoneoscopic extirpation of the nonfunctioning left renal unit was carried out uneventfully with a mean operating time, blood loss, and hospital stay of 124 minutes, 80 mL, and 3 days, respectively. There were no complications. The dissection in these cases was difficult, as the dysplastic calcified kidney was plastered in the retroperitoneum. Conclusion: Unilateral AMCDK with contralateral segmental dilation of the ureter may be separate entity or a coincidental finding, and it should not be confused with urinary tuberculosis unless there is microbiological and radiologic or histopathologic evidence of infection. Minimally invasive surgery in the form of retroperitoneoscopic extirpation is feasible, safe, and effective in such cases, although difficult, and it requires skills as well as experience.