The terms pigmentary mosaicism or patterned dyspigmentation describe a spectrum of clinical findings that range from localized areas of dyspigmentation with no systemic findings to widespread dyspigmentation with associated neurologic, musculoskeletal, and cardiac abnormalities, and other sequelae that can lead to early demise. Given this wide spectrum, these patients must be approached with caution, but with the understanding that most who have localized pigmentary anomalies, such as segmental pigmentary disorder (SegPD) seem to have no systemic manifestations. These patients can be approached in many different ways, but generally children with more widespread dyspigmentation, and any with associated abnormalities or not meeting neurodevelopmental milestones, should be evaluated closely. Children with any red flags warrant subspecialty referral, and all children deserve close clinical follow-up with their primary care physician to ensure they meet all of their developmental milestones. Fortunately, parents can be reassured that most children with SegPD, and many with more widespread patterned pigmentation, are otherwise healthy.