Occasionally, heterotopic splenic tissue can occur in the renal fossa secondary to splenosis following splenic trauma or splenectomy. Rarely, it can represent a developmental anomaly secondary to fusion of splenic and renal tissues. Splenorenal fusion can present as a renal mass, mimicking primary or secondary renal neoplasm on imaging studies, or with symptoms of hypersplenism (anaemia). To our knowledge, only seven cases of splenorenal fusion have been previously reported and the present case is only the second presentation in a young child. We report a case of splenorenal fusion in a 26-month-old girl with symptoms of hypersplenism and an abdominal mass associated with a ventricular septal defect, an umbilical hernia and a previous congenital dislocation of the hip.