We report on a case of cervical cutaneomeningospinal angiomatosis (Cobb's syndrome), a rare somatic disorder, characterized by vascular abnormalities of the spinal cord, with a triad of associated vascular skin, muscle, bone, and dura involvement at the same somite. This case follows an 18-year-old male patient presenting with left extremity weakness and back cervical pain. Magnetic resonance imaging (MRI) revealed a spinal cord arteriovenous malformation (AVM) at the C3-C5 level. Cobb's syndrome was diagnosed by coexistence of cutaneous naevi in a dermatomal pattern and neurological signs of a spinal cord lesion together with cervical MRI and angiography. The patient underwent a combination of staged endovascular embolization and microsurgical resection. Multiple biopsies of the mass including the skin, muscle, dura, and spinal cord at the same somite revealed that the lesions had a similar pathology. Post-operative immunohistochemical characterizations on specimen included CD31, smooth muscle actin (SMA), vascular endothelial growth factor (VEGF), and matrix metalloproteinase (MMP-9). The unique associations of somatic and spinal cord lesion as well as angiogenic and inflammatory factor expressions in all specimens are reported.