A one‐year‐old phenotypically female French Bulldog was referred for urinary incontinence. Physical examination disclosed the presence of hypertrophic clitoris. Computer tomography highlighted the presence of a left intramural ectopic ureter, in presence of a fully constituted female reproductive tract. Retrograde cystoscopy confirmed the presence of the ectopic ureter that was treated with cystoscopic‐guided laser ablation although incontinence persisted. Ovariohysterectomy and clitoridectomy were performed and histopathology of gonads was consistent with ovotestis. The subject's karyotype was defined as XX SRY‐negative and the case was diagnosed with a 78 XX (SRY‐negative) disorder of sexual development. A treatment with phenylpropanolamine led to the resolution of the incontinence. The association of such disorder with ectopic ureter and urinary incontinence has never been reported in dogs. Our findings suggest that the displacement of the ureter might be related to an incorrect secretion of sexual hormones in fetal life due to the presence of ovotestis.