Concurrent congenital fibrolipomatous hamartoma and congenital nevus of infancy: A syndromic or chance association

Congenital nevi and fibrolipomatous hamartoma are benign tumors of childhood, the latter being very uncommon. Fibrous hamartoma of infancy typically occurs in <2 years of life. The concurrence of these two lesions is extremely rare. We report a case of congenital fibrolipomatous hamartoma and congenital nevus of infancy in a 6-month-old male infant. Clinically, a suspicion of benign versus malignant lesion beneath the giant congenital nevus prompted its surgical removal. The histopathology confirmed it to be a compound lesion with benign melanocytic nevi and fascicles of spindle cells with eosinophilic cytoplasm representing hamartoma. Several types of melanocytic combined lesions have been noted with neuroectodermal and mesenchymal components. Sometimes, malignant soft-tissue neoplasm such as liposarcoma, rhabdomyosarcoma, and ganglioneuroblastoma do occur. This case report highlights the role of prompt surgical excision and histopathological examination.