Rhabdomyosarcoma (RMS) is a malignant, soft tissue neoplasm consisting of cells derived from the primitive mesenchyme that exhibit a profound tendency to myogenesis. Historically, the spindle cell and sclerosing variants were subcategorized under embryonal type RMS. Spindle cell/sclerosing RMS (S-ScRMS) was recently recognized in 2013 by the World Health Organization as a stand-alone entity. Current data support that certain S-ScRMS cases have a more aggressive clinical course with a reduction of long-term survival, and those found in the head and neck region often exhibit extensive local recurrence. Here, we highlight an aggressive clinical course of S-ScRMS by presenting a case of a 7-year-old male child who presented with complaints of swelling in left cheek since 15 to 20 days. It was associated with pain. He was diagnosed as S-ScRMS and received chemotherapy but the disease was aggressive and progressed on chemotherapy. He received radiation but still the disease was inoperable, hence was kept on supportive care.