A 2½-year-old male child born of a nonconsanguineous marriage with high parental age presented with macrodactyly, dysmorphic features, large disfiguring lipomas, small fibromas, a linear verrucous epidermal naevus and lymphangioma circumscriptum. X-ray of hand revealed macrodystrophia lipomatosa progressiva. A lipoma and lymphangioma circumscriptum were confirmed by histopathology. The cosmetically disfiguring lipomas were excised. This is the first case report from India with the characteristic macrodactyly. This could possibly be the first case of report of a mutational disorder following intake of carbimazole during pregnancy.