Aglomerular segmental hypoplasia of the kidney is a disease that is uncommon in the adult; the average age at appearance of symptoms is between ten and 15 years. A clear female predominance has been noted, and this disease is often misdiagnosed as chronic pyelonephritis. However, the radiological and pathological characteristics are different to the latter. The etiology of aglomerular segmental hypoplasia has not yet been determined. It is important to acquire further knowledge regarding this disease, as in certain cases this information could help to avoid unnecessary nephrectomy.