A 1-year-old female child, firstborn of second-degree consanguineous marriage, with global developmental delay, presented with three episodes of seizures. History of seizures at 2 months of age was not evaluated. No history was suggestive of infection and immunodeficiency. Dermatological examination revealed hypopigmentation of skin, silver gray hair over the scalp, eyebrows, and eyelashes, and a normal iris pigmentation [Figure 3]. Blood investigations, imaging studies, and peripheral smear were normal. Scalp hair microscopy revealed multiple irregular large clumps of melanin in the medulla of the hair shaft [Figure 3]. She was diagnosed as a case of Type 1 GS. She was treated conservatively and discharged.