The purpose was to describe the clinical findings, surgery and outcome of a bilateral descemetocele in a ferret (Mustela putorius furo), resulting from corneal dystrophy. A female ferret was diagnosed with a descemetocele in the left eye, as a result of corneal dystrophy with crystalline deposits, which spontaneously detached. Surgery was performed with a corneal graft and a conjunctival flap. No systemic disease was correlated with the corneal dystrophy. After 3 months, the ferret was presented with a descemetocele of the right eye, still due to corneal dystrophy. The same surgery as previously described was performed. The ferret recovered uneventfully from both surgeries and maintained vision from both eyes. This is the first report of corneal dystrophy in a ferret, and the first report of successful surgery on a descemetocele in this species. Surgery is the treatment of choice, and if performed quickly and adequately, has a good outcome.