In this report, we present a case of penile calciphylaxis, an extremely rare and serious condition occurring in association with dysregulation of systemic calcium metabolism in the setting of chronic renal impairment. Calciphylaxis can occur at various body sites and is associated with diffuse vascular calcifications in small and medium-sized arteries of the involved tissues. Penile calciphylaxis has a grim prognosis. Calciphylaxis is an important etiologic differential diagnosis for penile necrosis and penile pain in patient's being treated with dialysis for end-stage kidney disease. Diagnosis of penile calciphylaxis is possible via clinical and radiological evaluations. Medical management may alleviate symptoms; however surgical interventions may be necessary, and histological studies may allow for definitive classification.