The clinical signs and pathological lesions, consistent with Bovine Progressive Degenerative Myeloencephalopathy (BPDME), are described in a heifer. The animal was an 18-month-old Braunvieh-/Brown Swiss crossbreed with 50% Brown Swiss. Both the sire as well as the dam were related to the bull "B.". The only neurological signs were a posterior ataxia. Mobility and coordination of the front legs were normal. The CK activity of the cerebrospinal fluid was enhanced. There was a moderate to severe degeneration of the white matter consisting of marked axonal degradation and distension and degradation of myelin sheats in all parts of the spinal cord as well as in the medulla oblongata. The lesions were more severe in the descending than in the ascending tracts.