Waardenburg syndrome is a rare disease characterized by sensorineural deafness in association with pigmentary defects. Depending on additional symptoms, WS have been classified into four types. Waardenburg syndrome type 4, also called as Waardenburg Shah Syndrome is a very rare congenital disorder with astounding variable clinical expression, characterized by pigmentary abnormalities of the hair (A white forelock of hair, premature graying) and pigmentary changes of the iris such as heterochromia or homochromia irides, sensorineural deafness and Hirschsprung disease. Three genes have been bestowed so far in consociation with EDNRB, EDN3, and SOX10 genes. The pattern of inheritance is multifarious with the SOX10 mutation affiliation with autosomal dominant inheritance whereas the EDNRB and EDN3 genes are passed down in an autosomally recessive pattern.

<p class="abstract"><span lang="EN-IN">Schwannomas, also called as neurilemomas, are well-encapsulated, slowly growing tumors that arise from schwann cells of peripheral nerves. Clinically, schwannomas present as asymptomatic, slowly enlarging lateral neck masses and so they tend to present late. The nerve of origin is not often made until the time of surgery. Preoperative diagnosis is aided by imaging studies such as magnetic resonance imaging and computed tomography. The treatment of choice of schwannomas of the neck typically involves complete surgical resection. Rapidly growing tumors with evidence of invasion, or complete loss of nerve function should trigger complete surgical excision of the tumor. Here, we report a rare case of cervical vagal schwannoma in a 45 year old female who got admitted with the complaint of a firm, painless mass on the left side of the neck. The management of the case is discussed along with the relevant literature.</span></p>