A 39-year-old man presented with progressive gait disturbance and urinary incontinence. Medical history disclosed nephrolithiasis, dark urine, osteopenia and osteoarthrosis for 15 years. Examination disclosed crural spastic paraparesis with sensory level at T1 and hyperpigmentation of the sclerae (figure). Neuroimaging studies disclosed cervical spondylotic myelopathy and several discs prolapses. Homogentisic acid levels by HPLC were high in 24-hour urine.