Acute Soft Head Syndrome (ASHS) is an exceptionally rare complication of sickle cell disease (SCD), characterized by scalp pain and swelling secondary to subgaleal hematoma formation. Distinguishing its nonspecific clinical features, which often mimic other SCD-related complications, poses a significant diagnostic challenge. Imaging plays a crucial role in differentiating ASHS from other potential diagnoses and in evaluating the presence of coexisting intracranial hemorrhage, a common complication associated with ASHS. This report presents a rare case of ASHS in a patient with SCD, highlighting the diagnostic complexities and underscoring the essential role of imaging in its evaluation and management.