From 2001 to 2008, 119 pediatric cases of meningococcal disease caused by serogroup W were reported in France. They cases represented 4% of all meningococcal disease cases in children and were mostly in infants (54%). Meningitis occurred in 78 (66%) patients but differed by isolate. Isolates of the clonal complex sequence type-22 were associated with nonmeningeal presentation. Further investigations of clinical tropism of meningococcal W isolates are warranted.