Introduction: West syndrome (WS) comprises a classic triad of infantile spasms (ISs), hypsarrhythmia, and psychomotor delay. It is considered a rare epileptic encephalopathy typical to manifest in infancy or early childhood. Case description: This is a case report of a 6-year-old male child who was a known case of global developmental disorder (GDD) with structural WS. The child presented with typical dental findings and a history of ISs in clusters. The case report elucidates the dental treatment modalities followed, which emphasized preventive approaches and home care to complement professional care. Recall appointments were planned to reinforce preventive measures. Conclusion: Dental care for special needs patients can become more challenging in patients with severe intellectual disabilities, poor communication skills, and a diminished capacity to participate in even routine dental procedures. Thus, the major emphasis of oral health care in patients of WS should be on preventive measures. Clinical significance: This case report emphasizes the role of a pediatric dentist in the early diagnosis and management of oral health issues in a child with WS. Home care instructions for parents should focus on prevention and aid in inculcating an adequate home care regime to maintain good oral health status.