Rabies is an acute fatal viral encephalitis usually transmitted from animals to men following domestic and wild animal bites. Rabies is endemic in Iran. It is the most important zoonotic disease in the country. Here, we describe a case report of grey wolf rabies in Iran. One grey wolf in Chaharmahal and Bakhtiari province showed signs of rabies. Clinical signs were characterised by increased sensitivity, ferocity, restlessness, and depression is accompanied by lethargy. After a while, the animal died. The brain samples were taken from the wolf soon after death. The sample was refrigerated and transported fresh on ice to the laboratory. Fluorescent antibody technique (FAT) confirmed rabies infection in the wolf. Prevention and control of this fatal disease require a sensitive surveillance system to follow suspected animal and human rabies cases thoroughly through the improved reporting system, which contains the history of exposure, clinical examinations, symptoms, and laboratory results. Rapid and accurate diagnosis of rabies is very important due to its zoonotic and public health.