The clinical significance of 5-S-cysteinyldopa (5-S-CD), a major intermediate in melanin synthesis, was evaluated as a potential diagnostic tumor marker for uveal melanoma. Serum concentrations of 5-S-CD in 6 out of 7 patients with uveal melanoma in the absence of extraocular metastases were close to those of controls. In contrast, serum concentrations of 5-S-CD were found to be elevated in 3 patients with systemic metastases of melanoma. In addition, 5-S-CD in intraocular fluids, including both aqueous and vitreous humor, was elevated in patients with uveal melanoma regardless of the presence or absence of systemic metastases. These results suggest that 5-S-CD in the intraocular fluid may serve as a useful biochemical marker for the diagnosis of uveal melanoma.