To determine the presentation, diagnosis, progression, and family risk of fibrotic myopathy, a disease with marked breed predisposition in the German Shepherd Dog (GSD). 41 dogs prospectively recruited to the University of Wisconsin-Madison Comparative Genetics and Orthopedic Laboratory between November 2019 to August 2022. Medical records of dogs diagnosed with fibrotic myopathy were reviewed upon referral. The following data were recorded: sex, age, weight, regio interscapularis (withers) height, date of neutering, coat color and length, and age at fibrotic myopathy diagnosis. A pedigree was also obtained. In the study population, breeds included 37 GSDs, a Belgian Malinois, a Belgian Malinois cross, and 2 dogs with a GSD phenotype and no pedigree. Mean age at fibrotic myopathy diagnosis was 5.9 ± 2.0 years, and duration of lameness before diagnosis was 5.6 months and ranged from 0.75 to 18 months. Males were overrepresented at 61% of the study population. Inherited familial risk for fibrotic myopathy in the GSD was supported by pedigree analysis. This was the largest case series of fibrotic myopathy to date, providing a more comprehensive look at presentation and progression of the disease. The longer duration of lameness in bilaterally affected dogs likely represents disease progression rather than a more severe phenotype. Family history data support a genetic contribution to fibrotic myopathy, suggesting that further genetic investigation is warranted.