Primary intrahepatic rhabdomyosarcoma is extremely rare in children. Here, we describe a case of embryonal botryoid-type rhabdomyosarcoma in the liver of a 6-year-old boy. The patient presented with an abdominal mass and there were no abnormal findings on serological and biochemical testing. Preoperative imaging studies revealed a large mass in the left lobe of the liver and no lesions elsewhere in the abdomen. The tumor was removed via an extensive left hepatectomy. Histological examination confirmed a botryoid variant of embryonal rhabdomyosarcoma. Abdominal ultrasonography and computed tomography are valuable for preoperative planning and for post-surgical follow-up in cases of rhabdomyosarcoma. © 2006 Elsevier Ireland Ltd. All rights reserved.