Introduction: Intramuscular myxoma is a rare benign soft tissue tumor which usually affects large muscle groups in patients aged between forty and sixty years. It is composed of hypocellular myxoid tissue. Incidence of intramuscular myxoma varies from 0.1 to 0.3 per 100,000 individuals. The aim of this study: to present the process of diagnosis and treatment of a patient with the rare soft tissue tumor. Case description: A 41-year-old female came to the Orthopedic Clinic with a 2-year history of a lump on the lateral side of her right thigh. Noticeable growth of the lesion and pain complaints started after skiing injury 7 months before the examination. The magnetic resonance imaging test demonstrated encapsulated intramuscular mass which measured 6 x 2 x 2 cm located in Vastus lateralis muscle. Due to an increasing pain and a significant size of the lesion, the patient was qualified for an excisional biopsy. After an excision, the lesion was found on histopathology to be an intramuscular myxoma. The patient did not complain of any neurological deficits, walking disorders. The follow-up imaging examination 3 months after surgery show no recurrence and the further observation was planned.