We describe an uncommon presentation of focal dermal hypoplasia (FDH), or Goltz syndrome, in three consecutive generations. FDH is a rare multisystem disorder affecting mesodermal and ectodermal structures, with the skin, eyes, teeth, and musculoskeletal systems most commonly affected (1). FDH results from mutations in the PORCN gene (2, 3). Ninety five percent of cases arise from novo mutations, whereas 5% are hereditary with an X-linked dominant inheritance pattern (1, 4, 5). Patient 1 is an 8-month-old female born of non-consanguineous marriage who presented with diffuse alopecia of the scalp, linear hypopigmented, atrophic papules, and plaques with peripheral hyperpigmentation on the left hemiabdomen and right lateral leg along Blaschko's lines as well as syndactyly of the right second and third toes. Skin biopsy from the abdomen showed a thin epidermis with flattened rete ridges and massive dermal edema within collagen fibers and reactive capillaries. Family history was significant for similar skin lesions and bone deformities in her mother and grandmother. Patient 2 (patient 1’s mother) is a 17-year-old female with similar linear hypopigmented, atrophic plaques with peripheral hyperpigmentation on the abdomen and right axilla, syndactyly of the right hand, patchy alopecia of the scalp, microdontia, teeth fusion, enamel defects, verrucous papillomas in the axillae and onycholysis. Patient 3 (patient 1’s grandmother), presented with similar hypopigmented, atrophic plaques on the abdomen and left arm. The presentation of patient 2 and patient 3 illustrates this phenomenon and predicts that patient 1 may manifest additional features of FDH later in life and should be monitored by a multidisciplinary team including a dermatologist, orthopedist, ophthalmologist, and dentist.