Brachydactyly type B1 (BDB1; OMIN #113000) is caused by truncating mutations in the ROR2, whereas BDB2 is the result of mutations in NOG. As the patient described here presented with unilateral atypical BDB-like phenotype, sequencing of ROR2 and NOG was performed, revealing the nucleotide substitution c.G275A in the NOG gene resulting in the amino acid change p.G92E. This evidence concerns the gene ROR2 and brachydactyly type B1.