Background: Primary musculoskeletal tumors are rare but impose a significant health burden, mainly affecting young, active people, and posing risks of disability and death. Managing these tumors requires careful, staged, multidisciplinary approaches. In Iran, despite advances, issues, such as delayed diagnosis, unplanned initial surgeries, and poor referral pathways, remain. 
Objective: This study aimed to present a clinical algorithm based on the current evidence and specifics of Iran’s health system.      
Methods: We conducted a narrative review of authoritative scientific literature, international guidelines (National Comprehensive Cancer Network, European Society for Medical Oncology), and local epidemiologic studies in Iran. The focus was on integrating clinical, radiologic, and pathologic findings to achieve accurate diagnosis and to formulate appropriate treatment plans.
Results: Accurate differential diagnosis relies on identifying specific radiologic patterns and avoiding unnecessary biopsies of lesions exhibiting pathognomonic benign features, commonly referred to as “leave-me-alone lesions.” Osteosarcoma is the most prevalent malignant bone tumor in Iran and exhibits a bimodal age distribution. Biopsy should be the final step in diagnosis, ideally performed by the surgeon responsible for definitive treatment. Uninformed interventions on soft tissue masses without appropriate imaging may contaminate tissue planes and reduce the likelihood of limb-salvage surgery.
Conclusion: Establishing multidisciplinary teams at referral centers, educating general practitioners on red flag signs, and reforming the patient referral pathway are pivotal strategies for improving patient outcomes in Iran.