Unilateral or bilateral agenesis of the parotid gland is an uncommon condition with unclear aetiology. Only 22 cases of unilateral salivary agenesis have been reported excluding the present case. We present a case of a 4-year-old female child who presented with complaints of slight discoloration of her tongue and was referred for MRI to rule out any vascular malformation. Imaging revealed a complete absence of the right parotid gland. Hypertrophy of the sublingual gland and hypoplasia of the parotid gland on the opposite side was also noted, an unusual finding as the contralateral parotid showed compensatory hypertrophy in the other reported cases.

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