Introduction: We describe the case of a 10-year-old girl with an unusually severe and complicated episode of acute septic arthritis due to Streptococcus dysgalactiae, and systematically review the literature describing osteoarticular infections with this organism. Case Presentation: A 10-year-old girl with hereditary sensory and autonomic neuropathy type 5 and chronic (Charcot) arthropathy of the right knee presented with severe swelling and warmth of the right knee associated with 2 days of fever. She was ultimately diagnosed with septic arthritis of the right knee due to S. dysgalactiae. Her subsequent clinical course was abnormally prolonged and complicated, requiring an 8-week hospitalization, 7 arthrotomies and 8 months of total antimicrobial therapy. Conclusions: Group G Streptococci, including S. dysgalactiae, are unusual causes of septic arthritis. Our patients' clinical course was more severe than typically seen with more common osteoarticular pathogens. We conducted a systematic review of the literature on this condition, which demonstrated 7 previously reported cases of septic arthritis due to this pathogen in children, and also review the most current nomenclature and classification scheme for the organism.