Introduction Carvajal syndrome is characterized by woolly hair, striated palmoplantar keratoderma and left-sided ventricular cardiomyopathy. It is inherited as an autosomal recessive disorder due to a homozygous mutation in the gene coding for desmoplakin, which truncates the C-terminal of the protein and maps to chromosome 6p24. Signs and symptoms of Carvajal syndrome include: woolly hair that is present from birth, palmoplantar keratoderma that develops after infancy, follicular keratoses on elbows, knees, face, abdomen and lower limbs, clubbing of fingers and rarely mucosal lesions. The desmoplakin (DSP) abnormality can result in arrhythmogenic ventricular cardiomyopathy. Clinical Presentation A 2 month old male of Ecuadorian descent presented with oral ulcerations and poor feeding as reported by his mother. The oral lesions were noted at 2 weeks of age. Bilateral dorsal tongue and palatal erosions with sloughing were noted on oral examination. Skin excoriations were noted at sites of electrocardiogram leads. It was also noted that the child had sparse woolly hair that extended on to the forehead and had a hoarse cry. Intervention and Outcome Biopsies of the anterior dorsal tongue, lingual epiglottis, and duodenal, gastric, esophageal and rectosigmoid mucosa were performed. The tongue and epiglottis surface epithelium consisted of discohesive squamous epithelial cells with interspersed inflammation and bacterial colonies. Esophageal biopsy showed suprabasilar separation from underlying lamina propria. Direct immunofluorescence studies were negative. Whole Exome Sequence Analysis revealed patient was compound heterozygous for the c.7623delT and c.7623delG pathogenic variants in the DSP gene. Conclusion The mucosal lesions of this syndrome can present intraorally, and have a rather unique histopathology characterized by dyskeratosis and discohesion. Patients with this syndrome require regular cardiac evaluations as the cardiac issues are of paramount importance.