An unusual example of angiomyolipoma, which presented as a bulging mass on the right lumbar region of a newborn baby, is presented. The case described is the first report of a newborn with an unusually located angiomyolipoma. The most common predilection sites are the renal parenchyma and the retroperitoneum. However, most of the patients described are adults, and to the best of our knowledge no newborn patient has previously been reported in the literature.