The role of autoimmune mechanisms in idiopathic small fiber neuropathy (SFN) is not completely understood. Serum IgM binding to trisulfated disaccharide IdoA2S-GlcNS-6S (TS-HDS) and IgG to fibroblast growth factor receptor 3 were associated with sensory motor polyneuropathies and sensory neuronopathy among others. In this retrospective case review, we describe the clinical and laboratory findings of idiopathic SFN in a small cohort of pediatric patients. Eight children were diagnosed with SFN clinically and confirmed by reduced epidermal nerve fiber density. No involvement of large fibers was confirmed by clinical examination and electrophysiological tests. Possible triggering factors were infectious mononucleosis in 4 patients and human papilloma virus vaccination in 1 patient. Tilt table test was positive in 1 patient, and clinical autonomic dysfunctions were noted in 6 patients. Five patients had positive IgM against TS-HDS, 3 of whom had lower extremity predominant paresthesia. In conclusion, a high proportion of patients with idiopathic SFN in our cohort had a positive IgM TS-HDS antibody. © 2017 Wolters Kluwer Health, Inc. All rights reserved.