Background: A subgaleal hematoma (SGH) describes scalp bleeding in the potential space between the periosteum and the galea aponeurosis. This hematoma generally occurs after vacuum-assisted and forceps delivery, but may also be seen following head trauma. Despite its benign course, SGHs may complicate by life-threatening events.

Case description: We report a case of a 10-year-old male with Ehlers-Danlos syndrome presenting with scalp swelling following minor head trauma. On examination, a small swelling was observed in the occipital region. During the follow up, as the volume of subgaleal hematoma was increasing, we performed needle aspiration to achieve volume reduction, and dressed with a cap like bandage that wrapped and compressed scalp. The patient was hospitalized due to hemodynamic instability and a blood transfusion was performed. Due to extended usage of compressive bandage, a large area of scalp tissue became necrotic. The necrotic scalp tissue was debrided and reconstructed by plastic and reconstructive surgery. After surgery, another hematoma formed extending from the front of the ear to the ipsilateral neck caused facial paralysis, this hematoma was evacuated and a drain was placed. The patient was followed up for 1 year and no recurrent cephalhematoma was observed.

Conclusion: Ehlers-Danlos is a rarely encountered connective tissue syndrome, this case underscores the importance for neurosurgery physicians to recognize the potential catastrophes, these patients may present with following even minor injury.