PurposeTo describe the clinical presentation, management, and histopathological results of a congenital eccrine hidrocystoma.ObservationsA 4-year-old healthy boy presented with left upper eyelid ptosis and superonasal painless mass since birth. Computed tomography showed a hypodense cystic lesion located in the extraconal orbital compartment medially. Following complete excision, histopathology revealed an eccrine hidrocystoma.Conclusions and ImportanceOrbital eccrine hidrocystomas should be considered in the differential diagnosis of orbital cystic masses in the pediatric age group.