Assessment and management of a young person with a severe disability is multifaceted and complex. Variations of puberty can cause major concerns for parents and carers, with fears of imminent menstruation, peer and personal differences, concern for height outcome, as well as grief for a loss of childhood. Addressing physical, emotional, and social issues assists in optimizing outcomes. This article outlines specific evaluation and detailed management strategies for female and male pubertal problems in the context of disability, including treatments for extreme pubertal delay or acceleration, menstrual management at different ages, contraceptive issues, and sexual function and choices for both sexes.