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Myotonic dystrophy type 2 (DM2) is a progressive muscular dystrophy with multi-system manifestations and can affect functional mobility, gait, and balance. Currently, there are few reports of functional outcome measures in this population. This case describes the change in function detected by the High-level Mobility Assessment Tool (HiMAT) and 30-second Chair Stand Test (CST) in a high-functioning adult female with DM2 after physical therapy treatment. The patient’s chief complaint was muscular pain and fatigue that affected activities of daily living (ADLs). Multimodal physical therapy focused on neuromuscular re-education, balance and strength training, patient education, and moderate cardiorespiratory training. Improvements were observed in manual muscle testing and in single-limb standing balance. A nine-point total improvement was observed in HiMAT score, and the CST detected a 100% improvement in number of sit-to-stands. The patient returned to prior level of function and was able to resume ADLs and recreational activities without complaints of muscular pain or fatigue.