This study aimed to summarize the clinical and radiologic features of KD and discuss the treatment approaches. The clinical and radiological manifestations of four histologically confirmed KD cases were analyzed. Two neuroradiologists examined the imaging data to determine the location, size, number, margin, computed tomography (CT) density, signal intensity on magnetic resonance imaging (MRI), contrast enhancement patterns, and involvement of adjacent structures. The patients consisted of three males and one female (sex ratio 3:1), and a mean age of 43 years (range, 13–71 years). The initial symptom in patients was painless subcutaneous masses with a duration ranging from 1 to 180 months. Peripheral blood eosinophilia absolute count and serum immunoglobulin E (IgE) levels were increased in 3 patients. 3 cases had multiple lesions and 1 case had a solitary lesion, the lesion regions included retroauricular (n = 2), parotid region (n = 1), submandibular gland (n = 1), maxillofacial region (n = 1), and preauricular (n = 1). Most mass margins were ill-defined and presented with diffuse subcutaneous fat atrophy around them, patients tend to present with enlarged lymph nodes with marked enhancement. KD should be considered where patients present with painless subcutaneous masses in the head and neck region, along with peripheral blood eosinophilia and elevated serum IgE levels. Given its propensity for recurrence, prolonged follow-up is required.