This case report describes a rare fungal infection, piedra alba, in a 5-year-old female initially misdiagnosed. Treatment with 2 % ketoconazole shampoo led to significant regression within a week, without the need for hair cutting. We discuss the importance of early and accurate diagnosis, highlighting potential hair damage and complications in immunocompromised cases. Dermatoscopy aided diagnosis, and 2 % ketoconazole demonstrated efficacy, emphasizing the need for a multidisciplinary approach and dermatological follow-up.