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A 19-year-old male, right dominant, diagnosed with myotonic dystrophy (MD) Type I was referred to the rehabilitation services in a public hospital. The patient abstained from a job of shopkeeper for the past 3 months due to progressive hand function affectation. Functional evaluation revealed difficulty to release handheld objects and decreased grip strength. Hence, improving hand function was selected as therapeutic goal using client-centered approach. Six full-text articles were retrieved which could guide in planning disease-specific evaluation strategies and occupational therapy interventions. Purdue pegboard hand function test, grip strength using Jamar dynamometer, and pinch strength using pinchometer were performed pre- and post-4 weeks of intervention. Modified hand function training along with vocational counseling was selected as intervention strategy. Posttherapy, Purdue pegboard component scores on preferred hand, nonpreferred hand, and both hands and assembly scores showed 24%, 12%, 12%, and 8% improvement, respectively. Grip strength improved by 2 kg in the right and by 9 kg in the left hand on a dynamometer. Pinch strength improved by 1 kg bilaterally on pinchometer. Hence, Evidence-based clinical practice was found to be effective in the management of MD type I. [ABSTRACT FROM AUTHOR]