Pediatric neurodegenerative disorders are a group of rare diseases, often genetic in origin, arising from a variety of etiologies. Patients, their families, and their care providers face unique challenges in navigating expected growth and development with simultaneous loss of senses or learned skills. Juvenile-onset Huntington’s disease is an example of an inherited ultra-rare pediatric neurodegenerative disorder that exemplifies the difficulty diagnosing, treating, and communicating about pediatric neurodegenerative disorders. In particular, goals of care may change as the disease progresses, and there is no disease-modifying therapy or cure. A multidisciplinary team, including early involvement of palliative care, is important for providing compassionate, comprehensive care in pediatric neurodegenerative disorders.