Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy, is an unusual clinical entity. It classically presents with massive cervical lymphadenopathy, but atypical presentations have been described in the absence of nodal involvement, making diagnosis difficult. We report a case that presented with isolated involvement of the maxillary alveolus and discuss the diagnostic dilemmas and approaches to management in patients with atypical clinical features.