Owing to its rarity, rhabdomyosarcoma of the head and neck (HNRMS) has seldom 
been discussed in the literature. As most of the data is based only on the 
retrospective experiences of tertiary healthcare centers, there are difficulties 
in formulating a standard treatment protocol. Moreover, the disease is poorly 
understood at its pathological, genetic, and molecular levels. For instance, 20% 
of all histological assessment is inaccurate; even an experienced pathologist 
can confuse rhabdomyosarcoma (RMS) with neuroblastoma, Ewing's sarcoma, and 
lymphoma. RMS can occur sporadically or in association with genetic syndromes 
associated with predisposition to other cancers such as Li-Fraumeni syndrome and 
neurofibromatosis type 1 (von Recklinghausen disease). Such associations have a 
potential role in future gene therapies but are yet to be fully confirmed. 
Currently, chemotherapies are ineffective in advanced or metastatic disease and 
there is lack of targeted chemotherapy or biological therapy against RMS. Also, 
reported uses of chemotherapy for RMS have not produced reasonable responses in 
all cases. Despite numerous molecular and biological studies during the past 
three decades, the chemotherapeutic regimen remains unchanged. This vincristine, 
actinomycin, cyclophosphamide (VAC) regime, described in Kilman, et al. (1973) 
and Koop, et al. (1963), has achieved limited success in controlling the 
progression of RMS. Thus, the pathogenesis of RMS remains poorly understood 
despite extensive modern trials and more than 30 years of studies exploring the 
chemotherapeutic options. This suggests a need to explore surgical options for 
managing the disease. Surgery is the single most critical therapy for pediatric 
HNRMS. However, very few studies have explored the surgical management of 
pediatric HNRMS and there is no standard surgical protocol. The aim of this 
review is to explore and address such issues in the hope of maximizing the 
number of options available for young patients with HNRMS.