Primary RMS of the liver is a rare and commonly misdiagnosed entity due to its non-specific presentation and uncommon location. Lack of characteristic clinical parameters or imaging makes histopathology the gold standard for diagnosis [2]. Though no single specific tumor marker has been identified, lactate dehydrogenase (LDH) is widely used as it is reported to be a negative prognostic marker [3, 4].

The previous case reports showed a mean survival time of 9.25 mo (2–9) with a single report showing survival of 38 mo. This is an unique case reporting primary liver rhabdomyosarcoma with peritoneal disease with excellent survival. Of all hepatic RMS cases reported, the mean survival time of patients who underwent surgery is significantly better than those who were treated with non-surgical modalities. Most of the surgeries were anatomical resections (left/right hepatectomy or extended hepatectomy) based on the size and the site of the tumor, and there was no significant difference in surgical methods.

Tumor histology affects survival with embryonal subtypes having better prognosis than pleomorphic, spindle cell and alveolar varieties [5]. Further sub-types which differ in behavior and morphology were introduced based on molecular markers [6]. Malempati and Hawkin suggested risk stratification of RMS based on tumor size, invasiveness, nodal status, primary tumor site, pathological and PAX-fusion status [7].

Multi-modality management includes surgery, radiation therapy, and chemotherapy. Neoadjuvant chemotherapy not only helped to curtail metastatic spread but also to reduce the tumor size that improved future liver remnant and allowed complete tumor resection. Based on authors’ experience and previous case reports, multimodality management might be a key factor affecting survival.