Abstract The aim of this retrospective population-based study was to determine if there is any significant difference in academic achievement between students with a Pierre Robin Sequence (PRS) compared with the general population of Swedish students at the typical time of graduation from compulsory school (usually at 16 years of age). The data were obtained from the Swedish Medical Birth Register for the years 1973-1986 and linked to the Swedish School-Grade Register, which generated a data set of 68 individuals with PRS that were compared with a control group consisting of 1,249,404 individuals. The following outcomes were measured: not receiving a school leaving certificate; their odds of receiving lowest grade; and reduced odds of receiving high grade in the following subjects: mathematics; English; Swedish; and physical education, and their grade point average. For the control group 2.74% did not receive their leaving certificate, while for the individuals with PRS 9.68% (OR = 4.00; 95% CI = 1.81-8.86) did not receive their leaving certificate. There were no differences between the groups in mathematics, English, and Swedish, while, in physical education, individuals with PRS had significantly reduced odds of receiving a high grade in the relative grading system. Individuals with PRS had a significantly lower grade point average: 3.00 ± 0.09 in comparison to the control group: 3.23 ± 0.002, p = 0.002. This study indicates that individuals with PRS experience significant difficulties in their educational achievements in compulsory school in Sweden.