A 19-year-old woman with a condition since birth, comprising reticular hyperpigmentation, palmoplantar hyperkeratosis, dental anomalies, hypoplasia of dermatoglyphics and a slight yellowish hue to the nails is presented. Her father and grandfather were similarly affected. The reticular hyperpigmentation, although generalized, was more intense on the flexural areas where extensive milia formation was also observed. This case represents an unusual genodermatosis. The milia formation and the presence of normal perspiration indicate a special form of Naegeli-Franceschetti-Jadassohn (NFJ) syndrome, or a new entity close to the NFJ syndrome. The differential diagnosis from other congenital reticulate pigmentary disorders is discussed.