Porphobilinogen in urine is seen in inherited disorders like acute intermittent porphyria and congenital erythropoietic porphyria, as well as acquired conditions due to toxins like heavy metals, chemicals, and acute alcoholism. Elevated urinary porphobilinogen is a key marker of acute porphyria attacks. In some cases, patients with acute intermittent porphyria may excrete excess PBG even in remission, but levels rise significantly during an attack.