Purpose:The aim of this study was to describe a novel presentation of conjunctival smooth muscle hamartoma and review the histopathologic findings of this entity.Methods:A 17-year-old African American adolescent boy presented with a pink, nontender lesion of the right bulbar conjunctiva that did not improve with medical management. He had no previous medical or ocular history. The lesion was excised.Results:Histopathologic examination disclosed morphologically benign smooth muscle bundles within the substantia propria that stained positively for smooth muscle actin, vimentin, and desmin consistent with the diagnosis of a smooth muscle hamartoma.Conclusions:Although congenital smooth muscle hamartomas of the conjunctiva have been rarely reported in the literature, this is the first described case of a smooth muscle hamartoma presenting in adolescence in the bulbar conjunctiva. This lesion should be considered in the differential diagnosis for adolescents with similar appearing lesions.