Ectopic intrathoracic kidney is a rare congenital anomaly, representing less than 5% of all renal ectopias. Most cases are discovered in asymptomatic adult patients undergoing imaging exams for unrelated reasons. Less than twenty cases of thoracic kidneys in the infant group have been reported in the literature, mostly comprising asymptomatic patients. Herein, we report a case of an 18-month-old boy with recurrent pneumonia episodes who was found to have a right-sided intrathoracic ectopic kidney. A brief literature review addressing the pathogenesis, prognosis, and treatment of this condition is also presented.