Introduction: Rhabdomyosarcoma is a high-grade malignant neoplasm with skeletal muscle differentiation; a common soft tissue sarcoma in children but considered one of the rarest in adults. Case description: We report a case of 35-year-old male with a chronic productive cough and haemoptysis for five days. A CT scan of the nasopharynx revealed a blocked left maxillary and ethmoid sinus with bone destruction. These findings raised a suspicion of a tumour, and trans-nasal endoscopic sinus surgery was performed. Discussion: Nasal rhabdomyosarcoma is a rare adult malignant tumour. Most patients have lymphatic metastasis or skull base tumour infiltration at the time of the initial diagnosis and treatment, which poses a challenge to the diagnosis and management. Conclusion: Nasal acinar rhabdomyosarcoma, one of the histopathological types of rhabdomyosarcoma, has rapid disease progression and high mortality. Therefore, the treatment of rhabdomyosarcoma requires a combination of surgery, chemotherapy, radiotherapy, and immunotherapy to work together to achieve the best care for the patient.