The invention relates to formula powder for maple syrup urine disease and a preparation method of the formula powder. The formula powder is prepared from vegetable oil fatty powder, amino acid, fattyacid, algae oil, nucleotide, compound vitamin and a complex mineral through a dry method preparation process; in the formula powder, the amino acid is adopted, leucine, isoleucine and valine are removed, on the one hand, nutritional supplementation of a patient can be guaranteed, on the other hand, the contents of the leucine, the isoleucine and the valine in the body of the patient can be effectively controlled. According to the formula, nutritional ingredients are scientifically matched, and the solubleness and the stability are very good. The preparation method is simple and easy to operate, and suitable for large-scale industrial production.