We described a rare case of Salmonella enteritidis osteomyelitis of the tibia combined with arthritis of the ankle joint. A 4-year-old, otherwise healthy girl was hospitalized with 9-day history of fever, left leg pain, and diarrhea. On admission, her left lower leg and ankle were markedly reddened and swollen. Laboratory examinations disclosed a WBC of 16,300/microliter and a C-reactive protein of 15.6 mg/dl. A T2-weighed magnetic resonance image of the leg depicted a high intensity area around the left distal tibia and an extremely high intensity fluid in her left ankle joint, leading to our diagnosis of purulent osteomyelitis of the tibia and arthritis of the ankle joint. Salmonella enteritidis was isolated from ankle joint fluid and later aspirated bone marrow of the tibia, but not from blood or stool. Because of poor response to intravenous treatment with panipenem/betamipron for 2 days, she underwent drainage and washing of the joint fluid, and intramedullary administration of cefotaxime and ampicillin. She completely recovered without sequelae following treatment with sensitive antibiotics for 4 weeks in total. There has not been any relapse for more than 1.5 years. The authors also bibliographically surveyed the literature published from 1966 to 2002 and found 35 Japanese patients with Salmonella osteomyelitis. The present patient was the second case caused by Salmonella enteritidis in Japan. Septic arthritis is a rare complication, accounting for only 8% of the patients. Since Salmonella enteritidis has been a leading serotype among human isolates of Salmonella species during the past decade, it would be warranted to determine whether osteomyelitis due to this organism is likely to increase.