We present a case of retroperitoneal schwannoma in a patient with complete situs inversus. A 40-year-old man with complete situs inversus was referred for a retroperitoneal tumor. Abdominal CT and MRI revealed a 25-mm well-demarcated tumor between the inferior vena cava and abdominal aorta. We performed laparoscopic tumorectomy using 5 ports. Microscopically, the tumor consisted mainly of vaguely palisaded spindle cells containing nuclei mildly varying in size and shape. Immunohistochemical staining was positive for S-100 protein. The tumor was diagnosed as a schwannoma. This is the first case report of laparoscopic resection for retroperitoneal tumor in a patient with complete situs inversus in Japan.