The aim of this study was to investigate the twinning rates (TWRs) in isolates relative to the TWRs in the surrounding populations. It is not uncommon that the TWR shows extreme values (high or low rates) within isolated subpopulations. Starting from the isolated populations of the Åland Islands in Finland (high rates), we enlarged our studies to other isolated subpopulations in other countries: the island of Gotland (high rates), the county of Älvsborg located in the southwestern part of Sweden (low rates), and mountain villages in Norway. In our statistical analyses, we paid special attention to the robustness of the variance formula of the TWR and to alternative confidence intervals for the TWR. Particularly, we show how to obtain the most precise confidence intervals for the twinning rates. These statistical methods are crucial when the extreme TWRs within subpopulations are compared with the TWRs within the general population. One must decide whether the differences are real or caused by random fluctuations within the small isolates.