Desmoid tumors are rare, locally invasive soft-tissue tumors with unpredictable clinical behavior. Imaging plays a crucial role in their diagnosis, measurement of disease burden, and assessment of treatment response. However, desmoid tumors’ unique imaging features present challenges to conventional imaging metrics. The heterogeneous nature of these tumors, with a variable composition (fibrous, myxoid, or cellular), complicates accurate delineation of tumor boundaries and volumetric assessment. Furthermore, desmoid tumors can demonstrate prolonged stability or spontaneous regression, and biologic quiescence is often manifested by collagenization rather than bulk size reduction, making traditional size-based response criteria, such as Response Evaluation Criteria in Solid Tumors (RECIST), suboptimal. To overcome these limitations, advanced imaging techniques offer promising opportunities. Functional and parametric imaging methods, such as diffusion-weighted MRI, dynamic contrast-enhanced MRI, and T2 relaxometry, can provide insights into tumor cellularity and maturation. Radiomics and artificial intelligence approaches may enhance quantitative analysis by extracting and correlating complex imaging features with biological behavior. Moreover, imaging biomarkers could facilitate earlier detection of treatment efficacy or resistance, enabling tailored therapy. By integrating advanced imaging into clinical practice, it may be possible to refine the evaluation of disease burden and treatment response, ultimately improving the management and outcomes of patients with desmoid tumors.