The blue-rubber-bleb-nevus syndrome is an uncommon neurocutaneous disorder where scattered, bluish, rubbery nevi occur on the body surface. These nevi are frequently noticeable at birth. In addition to the skin lesions, vascular malformations of internal organs, which tend to bleed, are frequently present. However, well-documented central nervous system angiomata are rarely encountered. We present herein the case of a young adult with central nervous system involvement. Patient presented with a slowly progressive ataxia and brain stem signs. A large posterior fossa, and multiple smaller hemispheric angiomata were noted. Only a very few well-described symptomatic cases have been reported in the literature; we have reviewed them here. Unlike these cases, our patient with extensive nervous system involvement had a chronic, slowly progressive and nonfatal course.