Renal dysplasia has rarely been reported to be associated with situs inversus. Only 4 cases were reported previously with a spectrum of anomalies including bilateral renal dysplasia, situs inversus totalis, and pancreatic and hepatic fibrosis. Recently we encountered another case. A 22-year-old mother, gravida 1 para 0, was in her 23rd week of gestation when she was found to have oligohydramnios. A dead baby was delivered by extraovular induction. On autopsy, Potter's sequences including flattened, low-set ears, receding chin, upturned nose, and club hands and feet were noted. The posterior fontanel was obliterated. Internal examination showed situs inversus totalis, bilateral renal dysplasia, and agenesis of bilateral ureters. There was increased interstitial fibrosis in the pancreas. None of the family members was known to have any renal anomalies. Pinar and Rogers suggested a new syndrome when situs inversus totalis was combined with bilateral renal dysplasia as well as multisystem fibrosis. In our case, 2 major abnormalities, i.e., situs inversus totalis and bilateral renal dysplasia, were present though the increase of interstitial fibrosis within the pancreas was not as significant. This case is likely to be within the spectrum of anomalies mentioned by Pinar and Rogers.