The aim of this study is to examine anatomical, pathological and virological findings of combined malformations in a 1 day old, female, Holstein Friesian Calf that naturally and congenitally infected with Bovine Viral Diarrhea Virus (BVDV). Anatomo-pathologic findings consisted of Arnold-Chiari malformation (ACM), partial corpus callosum agenesis, sacrococygeal agenesis, epitheliogenesis imperfecta, anchylotic spondylose and arthrogryposis. Microscopically, perivascular infiltration, edema, neuronal degeneration, necrosis and focal gliosis were observed in the central nervous system (CNS), especially in brain. BVDV antigen was detected by Enzyme Linked Immunosorbent Assay (ELISA) method in both mother and calf At immunohistochemical examination, bovine viral diarrhea virus positive immunoreaction was detected in neurons, glial cells, purkinje cells and ependymal epithelial cells of CNS and pars nervosa cells of pituitary gland. Furthermore, immunopositive reactions were also observed in perivascularly infiltrated neutrophil leukocytes and lymphocytes. To the best of authors' knowledge, this is the first report of epitheliogenesis imperfecta and BVDV positive immunoreactions in pituitary cells in a calf with a congenital and natural infection.