Monilethrix is a rare inherited hair disorder characterized by fragile, beaded hair shafts that break easily, resulting in sparse or patchy hair growth. We present the case of a four-year-old Indian girl with complaints of sparse, coarse, easily pluckable hair over the occipital and biparietal area of the scalp since the age of two. Clinical examination revealed characteristic features of monilethrix. The diagnosis was further confirmed by trichoscopy and microscopy. The patient was initiated on 2% minoxidil for management. This case highlights the importance of recognizing the clinical and diagnostic features of monilethrix for accurate diagnosis and management due to its rare presentation and sporadic nature.