This article discusses the case of a 78-year-old female with painless gross hematuria. Chest X-rays showed dextrocardia with situs inversus, and whole-body computed tomography scanning showed a renal mass in the right renal pelvis, no evidence of metastasis, and mirror-image organs with left-to-right transposition, which resulted in a diagnosis of situs inversus totalis (SIT). A laparoscopic transperitoneoscopic right nephroureterectomy was scheduled. To our knowledge, our case is the second case of laparoscopic nephroureterectomy in renal pelvic urothelial carcinoma with SIT to be presented, but it is the first case of laparoscopic transperitoneoscopic nephroureterectomy. Laparoscopic transperitoneoscopic nephroureterectomy was successfully performed in a renal pelvic urothelial carcinoma patient with SIT with a correct description of renal vascularity and abdominal anatomy.