We report an unusual presentation of Salmonella endophthalmitis in a two-month-old Chinese girl with unilateral leukocoria. Ultrasonographic and neuroradiologic studies revealed an enlarging retinal mass and for this reason, enucleation was performed. Histopathology of the globe revealed endophthalmitis. Vitreous and stool cultures grew a Salmonella typhimurium. Clinically, it is very difficult to differentiate this condition from retinoblastoma. Systemic salmonellosis and endophthalmitis may progress slowly. This condition should be added to the differential diagnosis of leukocoria simulating retinoblastoma.