A ten-year-old boy was referred with an enlarging amelanotic iris tumor and secondary glaucoma in the left eye. After excisional biopsy it proved histopathologically to be an iris melanoma. The patient also had multiple nevi on his trunk, scalp, and buttocks suggestive of familial atypical mole melanoma (FAM-M) syndrome and confirmed histopathologically. To the authors' knowledge, this is the first report of iris melanoma in association with FAM-M syndrome. The young age at tumor onset is suggestive of a predisposing condition such as FAM-M syndrome. The association of uveal melanoma with FAM-M syndrome is discussed.