The invention relates to a ACVR1-Fc fusion protein, and a preparation method and application thereof. Specifically, the invention relates to the fusion protein ACVR1-Fc containing an ACVR1 element and an Fc element, a DNA sequence coding the fusion protein, a vector containing the coding sequence, a host cell containing the vector, a preparation method for the fusion protein, and application of the above-mentioned substances in prevention and / or treatment of diseases or symptoms (such as diseases, cancers and the like related to pleonosteosis) related to ACVR1 abnormity (e.g., ACVR1 mutation and / or overactivity). The fusion protein provided by the invention has stable expression, high yield and high activity (e.g., effective inhibition of ossification and cartilage differentiation) and is simple to purify, so the fusion protein can be effectively used for prevention and treatment of diseases or symptoms related to ACVR1 abnormity.