Oral manifestations of Langerhans cell histiocytosis in the pediatric population are unusual in their presentation, not frequently encountered by dental professionals, often missed or misdiagnosed, and may well be the first or only sign of the disease, which could be fatal in its severe form, thus making it critical that these diagnoses are not misgauged. Due to the rareness of this condition, clinically useful guidelines are unavailable for guiding oral health care professionals. Here, we present two cases: A 2.5-year-old female and an 11-month-old male of Asian origin, both of whom were diagnosed by pediatric dentists via oral manifestations. To minimize delay in diagnosis, an analytic roadmap is also presented to help pediatric dentists and other oral health care professionals diagnose this condition accurately. It will also help physicians from different specialties with an overview of appropriate referrals.