Despite well-known genetic defects, the pathophysiology of the tumorigenesis in these patients remains unknown. Recently, type 1 myotonic dystrophy and female sex, but not nucleotide repeat expansion size, were associated with increased risk of developing tumors.5 Interestingly, the nucleotide expansion in both type 1 myotonic dystrophy and type 2 myotonic dystrophy occur in the noncoding region of the DMPK and ZNF9 genes, respectively.1,3 This indicates that they are transcribed but do not alter the protein-coding portion of the gene. The mutant messenger RNA is sequestered in the nucleus and alters the normal function of RNA splicing factors, subsequently affecting numerous downstream targets. The proposed association for the increased risk of uveal melanoma in patients with myotonic dystrophy relates to the dysregulation of the Wnt/β-catenin signaling pathway.1 This pathway is involved in the inhibition of apoptosis and the promotion of cellular proliferation and migration, and it has been demonstrated to be altered in a wide spectrum of neoplasms present in patients with myotonic dystrophy, including uveal melanoma.1 β-Catenin is a cell membrane protein of normal cells, but it can also be expressed in the cytoplasm and nucleus of malignant cells. The inhibitor of DNA binding 2 (Id2), a transcriptional target of β-catenin, is highly expressed in normal uveal melanocytes. It encodes a downstream regulator of the Wnt/β-catenin pathway, and its downregulation has been associated with epithelioid cell differentiation and metastatic death in patients with uveal melanoma.6 Another study demonstrated immunofluorescent staining for β-catenin in 22 of 82 samples (26.8%) of primary uveal melanoma. A statistically significant correlation was found between strong immunoreactivity to β-catenin and absence of metastasis, with no patient dying from metastasis in that subgroup.7 Conversely, increased expression of β-catenin was determined in the plasma membranes of class 2 (aggressive-epithelioid type) uveal melanoma and related to poor prognosis.6 These contradictory results demonstrate that evidence is controversial regarding β-catenin expression as being implicated in the relationship between myotonic dystrophy and uveal melanoma in the same individual.