- LB30.00 Renal agenesis - A condition where one or both kidneys does not form (or develop) in utero. (Potter's syndrome)
- LB30.4 Oligomeganephronia - Oligomeganephronic renal hypoplasia is a severe developmental defect of both kidneys characterized by a reduced number of nephrons (the functional unit of the kidney), hypertrophic glomeruli with diameters twice the normal size, hypertrophic tubules and thickening of Bowman's capsule, occurring in the absence of a urinary tract malformation.
- LB30.6 Fusion anomaly of kidneys - The embryological, incomplete fusion of renal lobules and/or kidneys.
- LB30.60 Lobulated kidney - Any condition caused by incomplete fusion of the developing renal lobules during the antenatal period. This condition may be asymptomatic.
- LB30.61 Fused pelvic kidney - A condition caused by failure of the kidneys to correctly develop during the antenatal period. This condition is characterized by the presence of a single kidney, along the midline of the body. This condition may present with kidney stones, hydronephrosis, kidney infection, hematuria, or may be asymptomatic. Confirmation is through observation of a fused kidney by imaging.
- LB30.62 Horseshoe kidney - Horseshoe kidney is the most frequent renal fusion anomaly and is characterised by the union of the inferior poles of the two kidneys through an isthmus. Horseshoe kidney is in fact an anatomical anomaly rather than a disease, but it does lead to predisposition to certain conditions such as hydronephrosis, nephrolithiasis or pyelonephritis. One third of individuals with horseshoe kidney are asymptomatic, with the anomaly being discovered fortuitously during a radiological examination. Urogenital or renal vessel anomalies may be associated with the condition. For cases requiring treatment, various therapeutic approaches are available and choice of treatment depends on the associated pathology.
- LB30.7 Ectopic or pelvic kidney - A birth defect characterized by an abnormally positioned kidney; may be asymptomatic or result in urine blockage, infection or kidney stones.
- LB30.8 Medullary sponge kidney - A condition characterised by cystic or saccular dilatations of the medullary collecting ducts seen with radiocontrast filling. A predisposition to stones and associated often with renal tubular acidosis. There is no clear genetic predisposition.
- LB30.9 Multicystic renal dysplasia - A condition characterized by abnormal development of the kidney, specifically in which the abnormal kidney does not form a reniform structure but rather, a collection of non-communicating cysts, with no renal functional tissue.