The invention provides a recombinant adeno-associated virus-mediated X chromosome-linked myotubular myopathy treatment drug. A recombinant adeno-associated virus vector carries a myotubularin gene expression box containing human miR-142-3p and miR-122 target sequences. In-vivo experiments show that the recombinant adeno-associated virus vector can be efficiently introduced into a body to continuously and stably express myotubularin, prolong the survival time of an animal model and recover the growth and the development of the animal model. Results prompt that the recombinant adeno-associated virus vector is expected to be developed into the novel X chromosome-linked myotubular myopathy treatment drug.