Non-traumatic acute subdural hematoma (SDH) in patients with scleroderma is infrequently described in literature reviewing the neurologic disorders in scleroderma. We report a case of a patient with scleroderma complicated by severe pulmonary arterial hypertension (PAH), and a history of pulmonary embolism on warfarin who developed an SDH, requiring hemicraniectomy after initiating therapy with IV epoprostenol. The proposed mechanisms for SDH development and management strategy are discussed.