The c.892-34 locus of the patient’s PRKAR1A gene was mutated from G to T to yield a heterozygous GT genotype; the 34th base in the intron preceding coding exon 9 was mutated from G to T. This mutation may produce a mutation in the protein encoded by PRKAR1A and was the cause of the described case of CNC. Here, PRKAR1A is linked to Carney complex.