Whipple's disease is a rare, chronic, multisystem illness that is path
ologically characterized by the accumulation of macrophages in the inv
olved tissue that have a positive periodic acid-Schiff reaction. It is
typically seen in middle-aged white men, and only four cases involvin
g persons younger than 15 years of age have been reported. CNS Whipple
's disease without intestinal manifestations is rare; only six cases h
ave been reported in the literature, all involving adults. We report t
he case of a young boy with clinical, laboratory, radiographic, and pa
thological signs and symptoms consistent with CNS Whipple's disease wh
o responded to therapy with trimethoprimsulfamethoxazole.