Analysis of the effect of sex combination of previously born children in the family on fertility rates was performed for 363,373 Danish families comprising a total of 613,900 children, to address the questions of sex preference and combination preference. The fertility rates were stratified by parental age, period and latency time to the next child, and fertility rate ratios were estimated using multiplicative Poisson regression models. Our results demonstrate a strong preference for a balanced composition of sexes in Danish families. In families with two or three children the highest fertility rates were seen in families who had same-sexed children. The lowest fertility rates were in families with two children of identical sex followed by a child of the opposite sex. A moderate sex preference for girls was indicated by higher fertility rates in two-boy families than in two-girl families.