We report on an 8-year-old boy with oculocerebrorenal syndrome of Lowe (OCRL) involving renal acidosis, hypotonia, congenital cataracts, and growth retardation. Bone scintigraphy was performed to evaluate the patient's skeletal system. The scintigraphic findings were as follows: (1) absence of radioactivity over the epiphyseal growth plates, (2) increased uptake in the skull and extremities, (3) non-visualization of both kidneys, and (4) widening of the bony shafts of the extremities. We suggest that bone scintigraphy can demonstrate both structural and metabolic bony abnormalities in OCRL. (Tzu Chi Med J 2003; 15:195-198)