Leukonychia totalis, a rare condition characterized by complete whitening of the nails, presents an intriguing diagnostic challenge, particularly when it occurs in pediatric patients without an established family history. We discuss a case of an 8-year-old boy with leukonychia totalis, involving all fingernails but sparing his toenails, in the absence of familial predisposition. This report aims to discuss the etiopathogenesis, differential diagnosis, and diagnostic approach in pediatric leukonychia totalis.