Hemihypertrophy or hemihyperplasia is a condition in which there is asymmetrical overgrowth of the cranium, face, trunk, and limbs on one side of the body. It is characterized by hyperplasia of tissues rather than a hypertrophy. It is more commonly seen in females than in males and usually has the right side of the face involved. It may be an isolated finding or may be associated with a variety of syndromes. We report here a case of isolated non-syndromic right facial hemihypertrophy associated with diffuse warty pigmentation on the right side of the face, which is rarely reported. The clinical features, various radiological and imaging findings, and differential diagnoses to be considered are discussed in this case report.