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ABSTRACT. Machado G.A.C., Silva D.N., Fontes T.N., Requião J.G.T., Requião K.G., Madureira K.M., Muramoto C. & Peixoto T.C. [Chondrodysplasia telemark type in a mini cow - Case report.] Condrodisplasia tipo telemark em bovino miniatura - Relato de caso. Revista Brasileira de Medicina Veterinária, 38(Supl.1):70-74, 2016. Departamento de Anatomia, Patologia e Clínicas Veterinárias, Escola de Medicina Veterinária e Zootecnia, Universidade Federal da Bahia, Av. Adhemar de Barros, 500, Ondina, Salvador, BA 40170-110, Brasil. E-mail: email@example.com It is reported a case of chondrodysplasia telemark type in a mini cow. A female mini cow, 3 month-old was received at the Veterinary Hospital (HOSPMEV) of the Federal University of Bahia (UFBA), with congenital malformations and history of recurrent tympanites. On physical examination, defects compatible with disproportionate dwarfism were seen, such as shortened and rotated members, thickening of joints of the limbs, rounded big head, short snout, brachygnatia and kyphosis. After fourteen days hospitalized, the animal died and X-ray examinations, necropsy and histopathology were performed. Radiographic evaluation confirmed the skeletal malformations detected in the skull, thorax and limbs, showing a large skull, moderately shorter jaw and short and tortuous long bones (femur, humerus, radius and ulna). Necropsy showed scapulohumeral and carpometacarpal joints moderately thickened and large amount of synovial fluid was seen during the cut. Histological evaluation of the long bones revealed irregular epiphyseal plates, and multiple cysts in the hypertrophic zone and between the degenerated chondrocytes or calcified mineralization zone. Apparently, this is the first report of disproportionate dwarfism affecting an animal whose parents were primordial dwarves.