IntroductionCavernous hemangiomas of the orbit are benign vascular malformations, commonly encountered in adults. Although they are infrequent in pediatric population their diagnosis and course are of a great significance, mainly because they can cause visual disturbances such as amblyopia that can ensue, and secondarily due to their cosmetic and psychological effect. Special attention is required in follow up and treatment. Additionally, a systemic evaluation is necessary in order to discover asymptomatic lesions elsewhere in the body carrying a risk of complications.Case presentationThe authors describe the clinical course, diagnosis, therapeutic approach and prognosis of an infant with an orbital cavernous hemangioma accompanying intracranial lesions. A female infant 18 months of age, presented with a mass in the left upper eyelid, causing blepharoptosis. Preoperative magnetic resonance imaging and angiography of the brain and the orbits showed a hemangioma of the left upper eyelid and intracranial lesions to the left temporal fossa and the pons. At the age of 2 years and 8 months she was admitted again due to severe eyelid swelling, intense strong pain, exophthalmos and collateral ophthalmoplegia. Two operations were performed to remove the orbit mass. Histological examination, showed characteristics of cavernous hemangioma.ConclusionThe atypical presentation of cavernous orbital hemangioma with early infantile onset, merits attention.