Incontinentia pigmenti (IP; OMIM#308300) is a rare multisystem disorder with an incidence
of 0.7:100,000 live births. IP is rare and predominantly seen in females. Mutations in IKBKG gene (Xq28, GenBank: NM_003639.3, OMIM#300248) were reported as underlying cause of IP. IKBKG encode NFkB protein, which controls the expression of other genes involved in cell proliferation, immunity, and inflammation. Oro‑dental abnormalities have been documented in 50%–75% IP cases. We present a case report of a 16‑year‑old female with probable clinical IP, Arnold–Chiari malformation, hydrocephalus, delayed psychological development, and seizures. The IP hallmark feature: hyperpigmentation of the skin along the Blaschko’s lines was present only on the left side of the body. Left‑sided hemifacial and tongue hypertrophy were present, which have not been reported previously. Consistent with published reports, tooth size and shape discrepancies were present. However, unlike previous reports, discrepancies were prominent on affected versus unaffected side. This paper provides IP literature review, clinical considerations, and insight on management.