Calciphylaxis is a rare syndrome characterized by deposition of calcium salts in soft tissues and blood vessels with subsequent ischemia, necrosis of the skin and underlying areas. Here we provide a literature review on the history, etiology, pathogenesis, clinical presentation, diagnosis, risk factors, course, treatment, and prognosis of the disease under study. The main clinical manifestations of isolated calciphylaxis of the penis include formation of painful violet and bluish-red nodules and/or nodes characterized by round or irregular shape and localized mainly on the balanus. Later on, development of necrosis and ulcers of irregular shape is observed in these areas, which are then replaced by rough scars. The diagnosis can be established based on clinical presentation, medical history, and high levels of calcium and phosphorus in the blood serum. Calciphylaxis is extremely rare in patients with normal calcium level. The diagnosis is verified using pathological examination, which shows deposition of calcium salts in the skin and soft tissues. Here we report our own clinical observation of penile idiopathic calciphylaxis in a patient with normal blood levels of calcium and phosphorus.