Perosomus elumbis is an occasional congenital anomaly of cattle, swine, sheep, and dogs with unknown etiology. This congenital abnormality occurs in both sexes. A dead Holstein calf characterized by musculoskeletal and external genitalia abnormalities was referred to the large animal hospital of University of Tehran. Radiographic evaluation and subsequent dissection revealed that the vertebral column was truncated at the level of first lumbar vertebra (L1). Moreover, L2-L5, sacrum and coccygeal vertebrae were absent. The dorsum of the lumbosacral region contained only soft tissues. Urogenital tract was incomplete, and it contained agenesis of the ovaries, uterine tubes, cervix, and vulva concurrent with unilateral umbilical artery agenesis. Small and large intestine contained blind-ended sacs. No testes, scrotum, and penis were found. The intact ureter was attached to a thin-walled fluid fill sac. The laboratory finding showed that the pH of the fluid was 6 and contained hemoglobin, white blood cells, bacteria, a few red blood cells, oxalate crystalline, and epithelial cells. It was concluded that the collected fluid was urine. This is the first report of perosomus elumbis in a Holstein calf having a lot of visceral abnormalities in Iran.