Anterior sacral meningocele is a rare and often occult disorder that is important to diagnose. It usually occurs sporadically, and familial case reports are rare. This report describes ten documented cases of anterior sacral meningocele in one family. Spanning five generations, it is the largest such series ever reported. In most cases, computerized tomography (CT) was employed to make the diagnosis. There was a high incidence of associated anomalies. The mode of transmission appears to be autosomal dominant with variable penetrance, similar to several other reported series.