An 11-year-old girl was referred to the authors' hospital with a complaint of growth retardation. Physical examination revealed splenomegaly. Laboratory examination revealed increased sedimentation rate. Her imaging studies showed a splenic mass. Splenectomy was performed and histopathological examination revealed sclerosing angiomatoid nodular transformation (SANT) of the spleen. The disease rarely affects children but it could cause growth retardation and increased sedimentation rate, mimicking the chronic inflammatory diseases.