RESUMO
BACKGROUND: Cutaneomeningospinal angiomatosis or Cobb syndrome is a rare, not well understood phacomatosis that features metameric cutaneous and spinal arteriovenous malformations (AVMs). The first case was described in Boston in 1915, and since then, few more cases have been reported in the English literature. No case was found to be from Argentina. CASE DESCRIPTION: The authors present a 16-year-old boy with acute quadriparesis and respiratory failure who was diagnosed as Cobb syndrome and treated with microsurgery alone with very good results. CONCLUSION: Authors believe that microsurgery, alone or combined with embolization, should be the mainstay of treatment. They also acknowledge Harvey Cushing's contribution to the description of the syndrome and propose the syndrome to be renamed as Cobb-Cushing syndrome.