Studies on precocious puberty have primarily focused on children with typical patterns of growth and cognitive development. This study reviewed diagnostic data from the records of 15719 patients with neurodevelopmental disabilities for diagnoses associated with premature sexual development/precocious puberty. Thirty-two individuals with premature sexual development were identified, with the earliest changes seen in one girl at 1 year 7 months of age. In this group, the mean age at onset was 7 years 2 months in boys and 5 years 11 months in girls. Central precocious puberty, which was the most common cause of onset of early pubertal changes, was present in 15 of the 32 children. The results of this study suggest that children with a neurodevelopmental disability are at increased risk of premature pubertal changes when compared to children without a neurodevelopmental disability. This study indicates the need for health-care providers to be vigilant in screening for early pubertal changes in children with neurodevelopmental disabilities.