A pediatric patient diagnosed with Succinic Semialdehyde Dehydrogenase Deficiency required comprehensive dental treatment due to pain and impaired chewing. Due to the child’s non-cooperative behavior and medical condition, the decision was made to provide the necessary dental care under general anesthesia. The comprehensive dental rehabilitation included restorations and essential extractions. To the best of the authors’ knowledge, this is the first reported case of general anesthesia utilization in pediatric patients with Succinic Semialdehyde Dehydrogenase Deficiency, with no complications observed during induction, extubation, and post-operative recovery. Additionally, a novel, previously unreported pathogenic mutation of the Aldehyde Dehydrogenase 5 family member A1 gene (ALDH5A1) was identified through genetic analysis, contributing to the understanding of Succinic Semialdehyde Dehydrogenase Deficiency and the broader genetic disorder literature. Emphasizing the success of general anesthesia in addressing dental issues for Succinic Semialdehyde Dehydrogenase Deficiency patients, this case highlights the need for tailored approaches in the absence of established protocols. The authors’ findings enrich the knowledge in pediatric dentistry and genetic disorders, advocating for individualized care in unique medical situations.