TheNAA10gene encodes the catalytic subunit of the N-terminal acetyltransferase protein complex A (NatA), which is supposed to acetylate approximately 40% of the human proteins. After the advent of next-generation sequencing, more variants in theNAA10gene and Ogden syndrome (OMIM# 300855) have been reported. Individuals withNAA10-related syndrome have a wide spectrum of clinical manifestations and the genotype–phenotype correlation is still far from being confirmed. Here, we report a three years old Chinese girl carrying a heterozygousde novo NAA10[NM_003491: c. 247C > T, p. (Arg83Cys)] variant (dbSNP# rs387906701) (ClinVar# 208664) (OMIM# 300013.0010). The proband not only has some mild and common clinical manifestations, including dysmorphic features, developmental delay, obstructive hypertrophic cardiomyopathy, and arrhythmia, but also shows some rare clinical features such as exophthalmos, blue sclera, cutaneous capillary malformations, and adenoid hypertrophy. Our attempt is to expand the clinical phenotype associated withNAA10-related syndrome and explore genotype–phenotype correlation with such syndrome.