Systemic T-cell lymphoma presenting with neurologic symptoms is infrequently reported in immunocompetent children. We investigated the presenting features in all 20 immunocompetent children diagnosed with T-cell lymphoma at our institution from 1992-2004. Four children presented with neurologic features. These findings suggest that early neurologic involvement of T-cell lymphoma in immunocompetent children is underrecognized and that central nervous system involvement in these patients should be more thoroughly investigated.