Notably, we proved that the perturbation of DHX37 led to the upregulation of the β-catenin protein, which might underly the mechanism of DSD caused by defective DHX37. Our findings extend the variants associated with DSD and highlight the phenotype spectrum associated with DHX37. We also provided evidence that DSD caused by defective DHX37 may have a link with the activation of the Wnt/β catenin pathway. The gene discussed is DHX37; the disease is disorder of sexual differentiation.