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The management of polycystic kidney disease is mostly restricted to conservative measures. However, nephrectomy may be indicated in particular cases, especially when there are infective complications. To decrease the morbidity of the procedure, the laparoscopic approach has become appealing. We present a laparoscopic retroperitoneal approach to complicated polycystic kidney disease in a high-risk patient. CASERESPORT: We performed right retroperitoneal laparoscopic nephrectomy in a 39-year-old man who had autosomal polycystic kidney disease and had undergone heart transplantation. The immunosuppressed patient presented with severe flank pain, generalized signs of infection, and acute renal insufficiency. With the patient in the right lateral decubitus position, the retroperitoneal space was entered by the open technique, and the posterior pararenal space was developed with finger dissection. Five trocars were used. After the renal vessels had been secured and divided, the cysts were successively punctured, gradually shrinking the operative specimen. The kidney was placed in an Endo-catch and removed after morcellation, with no need to enlarge the 2-cm lumbotomy. The operating time was 80 minutes, and the hospital stay was 4 days. Histologic examination revealed a polycystic kidney with Aspergillus infection.The laparoscopic approach is a less-invasive option for removing a polycystic kidney when nephrectomy is indicated. The retroperitoneal route has the advantage of minimizing infection risks because of the absence of peritoneal opening.