Familial progressive hyper- and hypopigmentation (FPHH) is a rare genodermatosis characterized by multiple café au lait spots and hypopigmented ash-leaf macules intermingled with blotchy hyperpigmentation (1,2). Herein, we describe a Turkish FPHH patient with café-noir spots. A 16-year-old male presented to our Pediatric Dermatology Clinic, with extensive hyperpigmentation, irregular brown patches, and hypopigmented spots on his face, limbs, and trunk. He was born at term to healthy consanguineous parents with no history of pigmentary abnormalities. The lesions on the patient's perioral, periungual, and genital areas were noted at birth, and they did not increase in size or number. Patient anamnesis included a single episode of afebrile seizure during the neonatal period and a mild specific learning disability that required special education programming during elementary school.