ABSTRACT BACKGROUND: Intra-orbital hematoma is an eye disease that is usually attributed to trauma, neoplasm, acute sinusitis or vascular malformations. However, spontaneous hematoma is a rare condition and can be an early manifestation of hematological disorders. CASE REPORT: This is a case report on a 2-days old male neonate presented with proptosis of the right eye since the second day of birth. The pregnancy was term and the delivery was at health center by spontaneous vaginal delivery in which no instruments were used. He had no history of trauma and no bleeding from other sites. On physical examination, he had axial proptosis of the right eye of 26mm, injected conjunctiva and corneal ulcer with infiltrates. On complete blood count, white blood cell was 23.53x103, red blood cells= 3.96x106,   platelets = 279x103/µL, hemoglobin = 13.5g/dl, hematocrit = 38.7%. Coagulation profile test showed; prothrombin time = 5.1 with reference range of (10- 14 seconds); partial thromboplastin time = 24.9 reference range of (22-27seconds)   and international normalization ratio = 0.4 with reference range (0.8- 1.1). Ultrasound showed hematoma collection, and clotted blood was surgically drained with no other intra-operative finding. After the surgery, he took systemic and topical antibiotics, and progressively, the proptosis, conjunctival injection and corneal ulcer improved.CONCLUSION: In our case, we were not able to know the exact cause for the orbital hematoma. Although it is not common, we have to consider the possibility of spontaneous hematoma as a cause of sudden proptosis in neonates. KEY WORDS: Proptosis, orbital hematoma, Hemorrhagic disease of new born