Abstract
AIM: This case report presents a unique instance of Dandy-Walker syndrome (DWS) with notable oral manifestations, emphasizing the importance of interdisciplinary management in such complex cases. BACKGROUND: DWS is a rare congenital cystic malformation of the posterior cranial fossa. Clinical findings include delayed motor development in infancy, macrocephaly and hydrocephalus with bulging occiput, nystagmus, and ataxia. DWS is also associated with hypertelorism, cardiac, skeletal, renal, dental, and vertebral malformations. CASE DESCRIPTION: A 10-year-old male patient presented with typical neurological symptoms of DWS, including hydrocephalus, developmental delay, and motor skill impairment, alongside distinctive dental anomalies such as a high arched palate, malocclusion, and poor oral hygiene. The interdisciplinary approach involved collaboration between neurologists, pediatricians, and dental specialists to address both the neurological and oral health challenges. Following clinical examination and behavioral assessment, dental treatments were planned and completed under local anesthesia. Dental management included preventive care, root canal treatment (RCT), stainless steel crowns (SSC), and coronal restorations. CONCLUSION: The patient tolerated the procedure well with no complications during or after the treatment. Dental care for individuals with DWS should be tailored to their needs, with regular monitoring and maintenance. Alternative communication methods and caregiver support are essential. CLINICAL SIGNIFICANCE: DWS is a rare clinical entity. Understanding the unique challenges associated with DWS can help dental professionals provide better care and improve the overall health and quality of life for these patients. HOW TO CITE THIS ARTICLE: Banu SN, Raj SN, Anand A, et al. Unraveling Dandy-Walker Syndrome: A Case Report with Clinical Presentations and Management Insights. Int J Clin Pediatr Dent 2025;18(3):299-304.