Sinus histiocytosis is a well-recognized condition that mainly affects children in the first decade of life. Although this usually benign disease often features massive cervical lymphadenopathy, numerous extranodal sites of involvement can be seen. We describe a new presenting manifestation for sinus histiocytosis. A 13-month-old infant developed a rapidly growing unilateral epibulbar tumor that involved the cornea. A complete physical examination revealed only mild inguinal lymphadenopathy. A diagnosis of sinus histiocytosis was made by histologic examination of the lesion after surgical excision.