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This report describes a case of diffuse neurofibroma(DNF) that presented with spontaneous intratumoral hemorrhage. A 31-year-old man with cutaneous manifestations typical of neurofibromatosis type 1(NF1) was referred to hospital with a progressively expanding scalp swelling. Magnetic resonance imaging(MRI) showed an acute hematoma surrounded by a dense mass on the parietooccipital scalp. Plain skull radiographs and a computed tomography(CT) scan revealed bony defects in the occipital bone around the lambdoid suture and the skull base, including the sphenoid and petrous bones. The tumor extended to the upper cervical region and infiltrated through the fascia of the musculature. The tumor and adjacent soft tissue were highly vascular, which made surgical resection difficult. We describe the clinical, radiological and pathological features of this patient and discuss the surgical methods used to avoid a life-threatening hemorrhage during surgery.