Three neonatal Belgian white and red calves with a thickened and rigid skin, characterized by the presenceof scaly plates separated by deep reddened fissures were presented for necropsy. The least affectedcalf still had a normal hair coat, whilst the other two cases were alopecic and additionally displayed eclabium,ectropion and microtia. Based on these findings, ichthyosis fetalis bovis was diagnosed. Histologicalexamination of skin samples obtained after euthanasia revealed an exuberant orthokeratotic hyperkeratosisof the stratum corneum. In human harlequin ichthyosis, a disorder highly resembling bovineichthyosis fetalis, a defective lipid transport in keratinocytes caused by a single locus autosomal recessivegene defect has recently been identified as the cause of the disorder. Pedigree information of the calvescombined with additional information of three other calves of the same breed allowed the identificationof 3 disease carrying bulls and suggests an autosomal recessive inheritance of bovine ichthyosis fetalis. Asthere is currently no cure for this lethal disease, a proper breeding program is the only option to minimizethe incidence of ichthyosis in this specific cattle breed.